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Roscoe O. Brady - Publications

Affiliations: 
National Institutes of Health, Bethesda, MD 
Area:
hereditary metabolic disorders
Website:
http://www.ninds.nih.gov/news_and_events/news_articles/pressrelease_20081006_Brady.htm
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Year Citation  Score
2016 Long Y, Xu M, Li R, Dai S, Beers J, Chen G, Soheilian F, Baxa U, Wang M, Marugan JJ, Muro S, Li Z, Brady R, Zheng W. Induced Pluripotent Stem Cells for Disease Modeling and Evaluation of Therapeutics for Niemann-Pick Disease Type A. Stem Cells Translational Medicine. PMID 27484861 DOI: 10.5966/Sctm.2015-0373  0.333
2016 Kaneski CR, Brady RO, Hanover JA, Schueler UH. Development of a model system for neuronal dysfunction in Fabry disease. Molecular Genetics and Metabolism. PMID 27471012 DOI: 10.1016/J.Ymgme.2016.07.010  0.315
2015 Yang C, Wang H, Zhu D, Hong CS, Dmitriev P, Zhang C, Li Y, Ikejiri B, Brady RO, Zhuang Z. Mutant glucocerebrosidase in Gaucher disease recruits Hsp27 to the Hsp90 chaperone complex for proteasomal degradation. Proceedings of the National Academy of Sciences of the United States of America. 112: 1137-42. PMID 25583479 DOI: 10.1073/Pnas.1424288112  0.312
2015 Schiffmann R, Swift C, Wang X, Brady RO, Ries M. A prospective 10year study of individualized, intensified enzyme replacement therapy in advanced Fabry disease Molecular Genetics and Metabolism. 114: S103. DOI: 10.1016/J.Ymgme.2014.12.232  0.337
2014 El-Amouri SS, Dai M, Han JF, Brady RO, Pan D. Normalization and improvement of CNS deficits in mice with hurler syndrome after long-term peripheral delivery of BBB-targeted iduronidase. Molecular Therapy : the Journal of the American Society of Gene Therapy. 22: 2028-37. PMID 25088464 DOI: 10.1038/Mt.2014.152  0.308
2014 Mistry PK, Liu J, Sun L, Chuang WL, Yuen T, Yang R, Lu P, Zhang K, Li J, Keutzer J, Stachnik A, Mennone A, Boyer JL, Jain D, Brady RO, et al. Glucocerebrosidase 2 gene deletion rescues type 1 Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 111: 4934-9. PMID 24639522 DOI: 10.1073/Pnas.1400768111  0.347
2014 Dai M, Han J, El-Amouri SS, Brady RO, Pan D. Platelets are efficient and protective depots for storage, distribution, and delivery of lysosomal enzyme in mice with Hurler syndrome. Proceedings of the National Academy of Sciences of the United States of America. 111: 2680-5. PMID 24550296 DOI: 10.1073/Pnas.1323155111  0.304
2014 Barranger JA, Brady RO, Grabowski GA, Mankin H, Mistry PK, Weinreb NJ. Position statement: National Gaucher Foundation Medical Advisory Board, January 7, 2014. American Journal of Hematology. 89: 457-8. PMID 24488939 DOI: 10.1002/Ajh.23687  0.372
2014 Meng X, Shen J, Kong M, Brady R, Li RA, Schiffmann R. Abnormal intracellular calcium handling: a key pathogenic and therapeutic target of the cardiac manifestations in Fabry disease Molecular Genetics and Metabolism. 111: S77. DOI: 10.1016/J.Ymgme.2013.12.177  0.335
2013 Erba PA, Minichilli F, Giona F, Linari S, Dambrosia J, Pierini A, Filocamo M, Di Rocco M, Buffoni F, Brady RO, Mariani G. 99mTc-sestamibi scintigraphy to monitor the long-term efficacy of enzyme replacement therapy on bone marrow infiltration in patients with Gaucher disease. Journal of Nuclear Medicine : Official Publication, Society of Nuclear Medicine. 54: 1717-24. PMID 23990684 DOI: 10.2967/Jnumed.113.121871  0.367
2013 Wang D, El-Amouri SS, Dai M, Kuan CY, Hui DY, Brady RO, Pan D. Engineering a lysosomal enzyme with a derivative of receptor-binding domain of apoE enables delivery across the blood-brain barrier. Proceedings of the National Academy of Sciences of the United States of America. 110: 2999-3004. PMID 23382178 DOI: 10.1073/Pnas.1222742110  0.311
2013 Yang C, Rahimpour S, Lu J, Pacak K, Ikejiri B, Brady RO, Zhuang Z. Histone deacetylase inhibitors increase glucocerebrosidase activity in Gaucher disease by modulation of molecular chaperones. Proceedings of the National Academy of Sciences of the United States of America. 110: 966-71. PMID 23277556 DOI: 10.1073/Pnas.1221046110  0.306
2013 Brady RO, Yang C, Zhuang Z. An innovative approach to the treatment of Gaucher disease and possibly other metabolic disorders of the brain. Journal of Inherited Metabolic Disease. 36: 451-4. PMID 22814681 DOI: 10.1007/S10545-012-9515-9  0.356
2010 Lu J, Chiang J, Iyer RR, Thompson E, Kaneski CR, Xu DS, Yang C, Chen M, Hodes RJ, Lonser RR, Brady RO, Zhuang Z. Decreased glucocerebrosidase activity in Gaucher disease parallels quantitative enzyme loss due to abnormal interaction with TCP1 and c-Cbl. Proceedings of the National Academy of Sciences of the United States of America. 107: 21665-70. PMID 21098288 DOI: 10.1073/Pnas.1014376107  0.369
2010 Kaneski CR, Schiffmann R, Brady RO, Murray GJ. Use of lissamine rhodamine ceramide trihexoside as a functional assay for alpha-galactosidase A in intact cells. Journal of Lipid Research. 51: 2808-17. PMID 20526001 DOI: 10.1194/Jlr.D007294  0.336
2010 Meng XL, Shen JS, Kawagoe S, Ohashi T, Brady RO, Eto Y. Induced pluripotent stem cells derived from mouse models of lysosomal storage disorders. Proceedings of the National Academy of Sciences of the United States of America. 107: 7886-91. PMID 20385825 DOI: 10.1073/Pnas.1002758107  0.351
2010 Giannini EH, Mehta AB, Hilz MJ, Beck M, Bichet DG, Brady RO, West M, Germain DP, Wanner C, Waldek S, Clarke JT, Mengel E, Strotmann JM, Warnock DG, Linhart A. A validated disease severity scoring system for Fabry disease. Molecular Genetics and Metabolism. 99: 283-90. PMID 19951842 DOI: 10.1016/J.Ymgme.2009.10.178  0.305
2009 Enquist IB, Nilsson E, Bianco CL, Månsson JE, Ehinger M, Brady RO, Richter J, Karlsson S. Development of novel therapies in murine models for Gaucher disease Clinical Therapeutics. 31: S198-S199. DOI: 10.1016/S0149-2918(09)80022-2  0.307
2008 Di Rocco M, Giona F, Carubbi F, Linari S, Minichilli F, Brady RO, Mariani G, Cappellini MD. A new severity score index for phenotypic classification and evaluation of responses to treatment in type I Gaucher disease. Haematologica. 93: 1211-8. PMID 18603565 DOI: 10.3324/Haematol.12379  0.337
2008 Aerts JM, Groener JE, Kuiper S, Donker-Koopman WE, Strijland A, Ottenhoff R, van Roomen C, Mirzaian M, Wijburg FA, Linthorst GE, Vedder AC, Rombach SM, Cox-Brinkman J, Somerharju P, Boot RG, ... ... Brady RO, et al. Elevated globotriaosylsphingosine is a hallmark of Fabry disease. Proceedings of the National Academy of Sciences of the United States of America. 105: 2812-7. PMID 18287059 DOI: 10.1073/Pnas.0712309105  0.382
2008 Moore DF, Brady RO. Systems-biology approach to sphingolipid-storage disorders Future Lipidology. 3: 105-111. DOI: 10.2217/17460875.3.1.105  0.368
2007 Enquist IB, Lo Bianco C, Ooka A, Nilsson E, MÃ¥nsson JE, Ehinger M, Richter J, Brady RO, Kirik D, Karlsson S. Murine models of acute neuronopathic Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 104: 17483-8. PMID 17954912 DOI: 10.1073/Pnas.0708086104  0.371
2007 Shen JS, Meng XL, Schiffmann R, Brady RO, Kaneski CR. Establishment and characterization of Fabry disease endothelial cells with an extended lifespan. Molecular Genetics and Metabolism. 92: 137-44. PMID 17644384 DOI: 10.1016/J.Ymgme.2007.06.003  0.311
2007 Shin SH, Murray GJ, Kluepfel-Stahl S, Cooney AM, Quirk JM, Schiffmann R, Brady RO, Kaneski CR. Screening for pharmacological chaperones in Fabry disease. Biochemical and Biophysical Research Communications. 359: 168-73. PMID 17532296 DOI: 10.1016/J.Bbrc.2007.05.082  0.318
2007 Moore DF, Gelderman MP, Ferreira PA, Fuhrmann SR, Yi H, Elkahloun A, Lix LM, Brady RO, Schiffmann R, Goldin E. Genomic abnormalities of the murine model of Fabry disease after disease-related perturbation, a systems biology approach. Proceedings of the National Academy of Sciences of the United States of America. 104: 8065-70. PMID 17470787 DOI: 10.1073/Pnas.0701991104  0.344
2007 Schiffmann R, Askari H, Timmons M, Robinson C, Benko W, Brady RO, Ries M. Weekly enzyme replacement therapy may slow decline of renal function in patients with Fabry disease who are on long-term biweekly dosing. Journal of the American Society of Nephrology : Jasn. 18: 1576-83. PMID 17409308 DOI: 10.1681/Asn.2006111263  0.302
2007 Moore DF, Krokhin OV, Beavis RC, Ries M, Robinson C, Goldin E, Brady RO, Wilkins JA, Schiffmann R. Proteomics of specific treatment-related alterations in Fabry disease: a strategy to identify biological abnormalities. Proceedings of the National Academy of Sciences of the United States of America. 104: 2873-8. PMID 17301227 DOI: 10.1073/Pnas.0611315104  0.34
2007 Ries M, Kim HJ, Zalewski CK, Mastroianni MA, Moore DF, Brady RO, Dambrosia JM, Schiffmann R, Brewer CC. Neuropathic and cerebrovascular correlates of hearing loss in Fabry disease. Brain : a Journal of Neurology. 130: 143-50. PMID 17105746 DOI: 10.1093/Brain/Awl310  0.313
2007 Lonser RR, Schiffman R, Robison RA, Butman JA, Quezado Z, Walker ML, Morrison PF, Walbridge S, Murray GJ, Park DM, Brady RO, Oldfield EH. Image-guided, direct convective delivery of glucocerebrosidase for neuronopathic Gaucher disease. Neurology. 68: 254-61. PMID 17065591 DOI: 10.1212/01.Wnl.0000247744.10990.E6  0.372
2007 Yoshimitsu M, Higuchi K, Ramsubir S, Nonaka T, Rasaiah VI, Siatskas C, Liang SB, Murray GJ, Brady RO, Medin JA. Efficient correction of Fabry mice and patient cells mediated by lentiviral transduction of hematopoietic stem/progenitor cells. Gene Therapy. 14: 256-65. PMID 16929352 DOI: 10.1038/Sj.Gt.3302839  0.31
2006 Enquist IB, Nilsson E, Ooka A, MÃ¥nsson JE, Olsson K, Ehinger M, Brady RO, Richter J, Karlsson S. Effective cell and gene therapy in a murine model of Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 103: 13819-24. PMID 16954197 DOI: 10.1073/Pnas.0606016103  0.346
2006 Ries M, Clarke JT, Whybra C, Timmons M, Robinson C, Schlaggar BL, Pastores G, Lien YH, Kampmann C, Brady RO, Beck M, Schiffmann R. Enzyme-replacement therapy with agalsidase alfa in children with Fabry disease. Pediatrics. 118: 924-32. PMID 16950982 DOI: 10.1542/Peds.2005-2895  0.369
2006 Moore DF, Gelderman MP, Fuhrmann SR, Schiffmann R, Brady RO, Goldin E. Fabry disease and vascular risk factors: future strategies for patient-based studies and the knockout murine model. Acta Paediatrica (Oslo, Norway : 1992). Supplement. 95: 69-71. PMID 16720469 DOI: 10.1080/08035320600618932  0.354
2006 Brady RO. Emerging strategies for the treatment of hereditary metabolic storage disorders. Rejuvenation Research. 9: 237-44. PMID 16706651 DOI: 10.1089/Rej.2006.9.237  0.394
2006 Schiffmann R, Hauer P, Freeman B, Ries M, Scott LJ, Polydefkis M, Brady RO, McArthur JC, Wagner K. Enzyme replacement therapy and intraepidermal innervation density in Fabry disease. Muscle & Nerve. 34: 53-6. PMID 16583374 DOI: 10.1002/Mus.20550  0.326
2006 Ries M, Moore DF, Robinson CJ, Tifft CJ, Rosenbaum KN, Brady RO, Schiffmann R, Krasnewich D. Quantitative dysmorphology assessment in Fabry disease. Genetics in Medicine : Official Journal of the American College of Medical Genetics. 8: 96-101. PMID 16481892 DOI: 10.1097/01.Gim.0000200950.25118.Dd  0.345
2006 Brady RO. Enzyme replacement for lysosomal diseases. Annual Review of Medicine. 57: 283-96. PMID 16409150 DOI: 10.1146/Annurev.Med.57.110104.115650  0.355
2006 Schiffmann R, Ries M, Timmons M, Flaherty JT, Brady RO. Long-term therapy with agalsidase alfa for Fabry disease: safety and effects on renal function in a home infusion setting. Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association. 21: 345-54. PMID 16204287 DOI: 10.1093/Ndt/Gfi152  0.349
2005 Lee KO, Luu N, Kaneski CR, Schiffmann R, Brady RO, Murray GJ. Improved intracellular delivery of glucocerebrosidase mediated by the HIV-1 TAT protein transduction domain. Biochemical and Biophysical Research Communications. 337: 701-7. PMID 16223608 DOI: 10.1016/J.Bbrc.2005.05.207  0.32
2005 de Grey AD, Alvarez PJ, Brady RO, Cuervo AM, Jerome WG, McCarty PL, Nixon RA, Rittmann BE, Sparrow JR. Medical bioremediation: prospects for the application of microbial catabolic diversity to aging and several major age-related diseases. Ageing Research Reviews. 4: 315-38. PMID 16040282 DOI: 10.1016/J.Arr.2005.03.008  0.31
2005 Lonser RR, Walbridge S, Murray GJ, Aizenberg MR, Vortmeyer AO, Aerts JM, Brady RO, Oldfield EH. Convection perfusion of glucocerebrosidase for neuronopathic Gaucher's disease. Annals of Neurology. 57: 542-8. PMID 15786474 DOI: 10.1002/Ana.20444  0.361
2005 Ries M, Gupta S, Moore DF, Sachdev V, Quirk JM, Murray GJ, Rosing DR, Robinson C, Schaefer E, Gal A, Dambrosia JM, Garman SC, Brady RO, Schiffmann R. Pediatric Fabry disease. Pediatrics. 115: e344-55. PMID 15713906 DOI: 10.1542/Peds.2004-1678  0.357
2005 Yoshimitsu M, Siatskas C, Liang S, Rasaiah VI, Higuchi K, Murray GJ, Takenaka T, Tei C, Brady RO, Medin JA. Long-Term and Sustained Correction of the α-Galactosidase A Deficiency in Fabry Mice and Patient Cells Receiving Lentivirally Transduced Hematopoietic Stem/Progenitor Cells. Blood. 106: 1285-1285. DOI: 10.1182/Blood.V106.11.1285.1285  0.332
2004 Schueler UH, Kolter T, Kaneski CR, Zirzow GC, Sandhoff K, Brady RO. Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease. Journal of Inherited Metabolic Disease. 27: 649-58. PMID 15669681 DOI: 10.1023/B:Boli.0000042959.44318.7C  0.354
2004 Brady RO, Schiffmann R. Enzyme-replacement therapy for metabolic storage disorders. The Lancet. Neurology. 3: 752-6. PMID 15556808 DOI: 10.1016/S1474-4422(04)00938-X  0.378
2004 Yoshimitsu M, Sato T, Tao K, Walia JS, Rasaiah VI, Sleep GT, Murray GJ, Poeppl AG, Underwood J, West L, Brady RO, Medin JA. Bioluminescent imaging of a marking transgene and correction of Fabry mice by neonatal injection of recombinant lentiviral vectors. Proceedings of the National Academy of Sciences of the United States of America. 101: 16909-14. PMID 15550536 DOI: 10.1073/Pnas.0407572101  0.315
2004 Moore DF, Ye F, Brennan ML, Gupta S, Barshop BA, Steiner RD, Rhead WJ, Brady RO, Hazen SL, Schiffmann R. Ascorbate decreases Fabry cerebral hyperperfusion suggesting a reactive oxygen species abnormality: an arterial spin tagging study. Journal of Magnetic Resonance Imaging : Jmri. 20: 674-83. PMID 15390234 DOI: 10.1002/Jmri.20162  0.335
2004 Ries M, Bettis KE, Choyke P, Kopp JB, Austin HA, Brady RO, Schiffmann R. Parapelvic kidney cysts: a distinguishing feature with high prevalence in Fabry disease. Kidney International. 66: 978-82. PMID 15327390 DOI: 10.1111/J.1523-1755.2004.00846.X  0.345
2004 Desnick RJ, Brady RO. Fabry disease in childhood. The Journal of Pediatrics. 144: S20-6. PMID 15126980 DOI: 10.1016/J.Jpeds.2004.01.051  0.375
2004 Kim EY, Hong YB, Lai Z, Kim HJ, Cho YH, Brady RO, Jung SC. Expression and secretion of human glucocerebrosidase mediated by recombinant lentivirus vectors in vitro and in vivo: implications for gene therapy of Gaucher disease. Biochemical and Biophysical Research Communications. 318: 381-90. PMID 15120612 DOI: 10.1016/J.Bbrc.2004.04.040  0.329
2004 Kim EY, Hong YB, Lai Z, Brady RO, Jung S. 863. Expression and Secretion of Human Glucocerebrosidase Mediated by Recombinant Lentivirus Vectors In Vitro and In Vivo Molecular Therapy. 9. DOI: 10.1016/J.Ymthe.2004.06.770  0.332
2003 Brady RO. Gaucher and Fabry diseases: from understanding pathophysiology to rational therapies. Acta Paediatrica (Oslo, Norway : 1992). Supplement. 92: 19-24. PMID 14989461 DOI: 10.1111/J.1651-2227.2003.Tb00215.X  0.356
2003 Matsuda J, Suzuki O, Oshima A, Yamamoto Y, Noguchi A, Takimoto K, Itoh M, Matsuzaki Y, Yasuda Y, Ogawa S, Sakata Y, Nanba E, Higaki K, Ogawa Y, Tominaga L, ... ... Brady RO, et al. Chemical chaperone therapy for brain pathology in G(M1)-gangliosidosis. Proceedings of the National Academy of Sciences of the United States of America. 100: 15912-7. PMID 14676316 DOI: 10.1073/Pnas.2536657100  0.359
2003 Schiffmann R, Floeter MK, Dambrosia JM, Gupta S, Moore DF, Sharabi Y, Khurana RK, Brady RO. Enzyme replacement therapy improves peripheral nerve and sweat function in Fabry disease. Muscle & Nerve. 28: 703-10. PMID 14639584 DOI: 10.1002/Mus.10497  0.346
2003 Brady RO. Enzyme replacement therapy: conception, chaos and culmination. Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 358: 915-9. PMID 12803925 DOI: 10.1098/Rstb.2003.1269  0.391
2003 Park J, Murray GJ, Limaye A, Quirk JM, Gelderman MP, Brady RO, Qasba P. Long-term correction of globotriaosylceramide storage in Fabry mice by recombinant adeno-associated virus-mediated gene transfer. Proceedings of the National Academy of Sciences of the United States of America. 100: 3450-4. PMID 12624185 DOI: 10.1073/Pnas.0537900100  0.308
2003 Desnick RJ, Brady R, Barranger J, Collins AJ, Germain DP, Goldman M, Grabowski G, Packman S, Wilcox WR. Fabry disease, an under-recognized multisystemic disorder: Expert recommendations for diagnosis, management, and enzyme replacement therapy Annals of Internal Medicine. 138: 338-346. PMID 12585833 DOI: 10.7326/0003-4819-138-4-200302180-00014  0.365
2002 Schueler U, Kaneski C, Murray G, Sandhoff K, Brady RO. Uptake of mannose-terminal glucocerebrosidase in cultured human cholinergic and dopaminergic neuron cell lines. Neurochemical Research. 27: 325-30. PMID 11958535 DOI: 10.1023/A:1014915430398  0.373
2002 Schiffmann R, Brady RO. New prospects for the treatment of lysosomal storage diseases. Drugs. 62: 733-42. PMID 11929328 DOI: 10.2165/00003495-200262050-00002  0.378
2002 Moore DF, Altarescu G, Ling GS, Jeffries N, Frei KP, Weibel T, Charria-Ortiz G, Ferri R, Arai AE, Brady RO, Schiffmann R. Elevated cerebral blood flow velocities in Fabry disease with reversal after enzyme replacement. Stroke; a Journal of Cerebral Circulation. 33: 525-31. PMID 11823664 DOI: 10.1161/Hs0202.102601  0.335
2001 Brady RO, Murray GJ, Moore DF, Schiffmann R. Enzyme replacement therapy in Fabry disease. Journal of Inherited Metabolic Disease. 24: 18-24; discussion 11. PMID 11758675 DOI: 10.1023/A:1012451320105  0.362
2001 Germain DP, Kaneski CR, Brady RO. Mutation analysis of the acid beta-glucosidase gene in a patient with type 3 Gaucher disease and neutralizing antibody to alglucerase. Mutation Research. 483: 89-94. PMID 11600137 DOI: 10.1016/S0027-5107(01)00232-9  0.372
2001 Brady RO. Tay-Sachs disease: the search for the enzymatic defect. Advances in Genetics. 44: 51-60. PMID 11596998 DOI: 10.1016/S0065-2660(01)44070-3  0.35
2001 Moore DF, Scott LT, Gladwin MT, Altarescu G, Kaneski C, Suzuki K, Pease-Fye M, Ferri R, Brady RO, Herscovitch P, Schiffmann R. Regional cerebral hyperperfusion and nitric oxide pathway dysregulation in Fabry disease: reversal by enzyme replacement therapy. Circulation. 104: 1506-12. PMID 11571244 DOI: 10.1161/Hc3801.096352  0.321
2001 Schiffmann R, Kopp JB, Austin HA, Sabnis S, Moore DF, Weibel T, Balow JE, Brady RO. Enzyme replacement therapy in Fabry disease: a randomized controlled trial. Jama. 285: 2743-9. PMID 11386930 DOI: 10.1001/Jama.285.21.2743  0.307
2001 Altarescu G, Hill S, Wiggs E, Jeffries N, Kreps C, Parker CC, Brady RO, Barton NW, Schiffmann R. The efficacy of enzyme replacement therapy in patients with chronic neuronopathic Gaucher's disease. The Journal of Pediatrics. 138: 539-47. PMID 11295718 DOI: 10.1067/Mpd.2001.112171  0.789
2001 Qin G, Takenaka T, Telsch K, Kelley L, Howard T, Levade T, Deans R, Howard BH, Malech HL, Brady RO, Medin JA. Preselective gene therapy for Fabry disease. Proceedings of the National Academy of Sciences of the United States of America. 98: 3428-33. PMID 11248095 DOI: 10.1073/Pnas.061020598  0.351
2001 Jung SC, Han IP, Limaye A, Xu R, Gelderman MP, Zerfas P, Tirumalai K, Murray GJ, During MJ, Brady RO, Qasba P. Adeno-associated viral vector-mediated gene transfer results in long-term enzymatic and functional correction in multiple organs of Fabry mice. Proceedings of the National Academy of Sciences of the United States of America. 98: 2676-81. PMID 11226298 DOI: 10.1073/Pnas.051634498  0.346
2000 Brady RO, Schiffmann R. Clinical features of and recent advances in therapy for Fabry disease. Jama. 284: 2771-5. PMID 11105184 DOI: 10.1001/Jama.284.21.2771  0.369
2000 Altarescu G, Schiffmann R, Parker CC, Moore DF, Kreps C, Brady RO, Barton NW. Comparative efficacy of dose regimens in enzyme replacement therapy of type I Gaucher disease. Blood Cells, Molecules & Diseases. 26: 285-90. PMID 11042029 DOI: 10.1006/Bcmd.2000.0310  0.765
2000 Takenaka T, Murray GJ, Qin G, Quirk JM, Ohshima T, Qasba P, Clark K, Kulkarni AB, Brady RO, Medin JA. Long-term enzyme correction and lipid reduction in multiple organs of primary and secondary transplanted Fabry mice receiving transduced bone marrow cells. Proceedings of the National Academy of Sciences of the United States of America. 97: 7515-20. PMID 10840053 DOI: 10.1073/Pnas.120177997  0.353
2000 Brady RO. Neurogene therapy for the 21st century. Archives of Neurology. 57: 54. PMID 10634437 DOI: 10.1001/Archneur.57.1.54  0.423
2000 Schiffmann R, Murray GJ, Treco D, Daniel P, Sellos-Moura M, Myers M, Quirk JM, Zirzow GC, Borowski M, Loveday K, Anderson T, Gillespie F, Oliver KL, Jeffries NO, Doo E, ... ... Brady RO, et al. Infusion of alpha-galactosidase A reduces tissue globotriaosylceramide storage in patients with Fabry disease. Proceedings of the National Academy of Sciences of the United States of America. 97: 365-70. PMID 10618424 DOI: 10.1073/Pnas.97.1.365  0.351
1999 Goldin E, Cooney A, Kaneski CR, Brady RO, Schiffmann R. Mucolipidosis IV consists of one complementation group. Proceedings of the National Academy of Sciences of the United States of America. 96: 8562-6. PMID 10411915 DOI: 10.1073/Pnas.96.15.8562  0.325
1999 Takenaka T, Hendrickson CS, Tworek DM, Tudor M, Schiffmann R, Brady RO, Medin JA. Enzymatic and functional correction along with long-term enzyme secretion from transduced bone marrow hematopoietic stem/progenitor and stromal cells derived from patients with Fabry disease. Experimental Hematology. 27: 1149-59. PMID 10390190 DOI: 10.1016/S0301-472X(99)00050-8  0.338
1999 Ohshima T, Schiffmann R, Murray GJ, Kopp J, Quirk JM, Stahl S, Chan CC, Zerfas P, Tao-Cheng JH, Ward JM, Brady RO, Kulkarni AB. Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice. Proceedings of the National Academy of Sciences of the United States of America. 96: 6423-7. PMID 10339603 DOI: 10.1073/Pnas.96.11.6423  0.333
1999 Zirzow GC, Sanchez OA, Murray GJ, Brady RO, Oldfield EH. Delivery, distribution, and neuronal uptake of exogenous mannose-terminal glucocerebrosidase in the intact rat brain. Neurochemical Research. 24: 301-5. PMID 9972879 DOI: 10.1023/A:1022578424693  0.387
1998 YOKOYAMA M, TRAMS EG, BRADY RO. Sphingolipid antibodies in sera of animals and patients with central nervous system lesions. Proceedings of the Society For Experimental Biology and Medicine. Society For Experimental Biology and Medicine (New York, N.Y.). 111: 350-2. PMID 14002620 DOI: 10.3181/00379727-111-27789  0.313
1998 TRAMS EG, BRADY RO. Cerebroside synthesis in Gaucher's disease. The Journal of Clinical Investigation. 39: 1546-50. PMID 13777865 DOI: 10.1172/Jci104175  0.309
1998 Argoff CE, Barton NW, Brady RO, Ziessman HA. Gastrointestinal symptoms and delayed gastric emptying in Fabry's disease: response to metoclopramide. Nuclear Medicine Communications. 19: 887-91. PMID 10581595 DOI: 10.1097/00006231-199809000-00009  0.75
1998 Dunbar CE, Kohn DB, Schiffmann R, Barton NW, Nolta JA, Esplin JA, Pensiero M, Long Z, Lockey C, Emmons RV, Csik S, Leitman S, Krebs CB, Carter C, Brady RO, et al. Retroviral transfer of the glucocerebrosidase gene into CD34+ cells from patients with Gaucher disease: in vivo detection of transduced cells without myeloablation. Human Gene Therapy. 9: 2629-40. PMID 9853529 DOI: 10.1089/Hum.1998.9.17-2629  0.759
1998 Brady RO. Therapy for the sphingolipidoses. Archives of Neurology. 55: 1055-6. PMID 9708954 DOI: 10.1001/Archneur.55.8.1055  0.367
1997 Brady RO. Gaucher's disease: past, present and future. Bailliã¨Re's Clinical Haematology. 10: 621-34. PMID 9497855 DOI: 10.1016/S0950-3536(97)80031-5  0.388
1997 Brady RO, Murray GJ, Oliver KL, Leitman SF, Sneller MC, Fleisher TA, Barton NW. Management of neutralizing antibody to Ceredase in a patient with type 3 Gaucher disease. Pediatrics. 100: E11. PMID 9382912 DOI: 10.1542/Peds.100.6.E11  0.78
1997 Schiffmann R, Heyes MP, Aerts JM, Dambrosia JM, Patterson MC, DeGraba T, Parker CC, Zirzow GC, Oliver K, Tedeschi G, Brady RO, Barton NW. Prospective study of neurological responses to treatment with macrophage-targeted glucocerebrosidase in patients with type 3 Gaucher's disease. Annals of Neurology. 42: 613-21. PMID 9382473 DOI: 10.1002/Ana.410420412  0.785
1997 Ohshima T, Murray GJ, Swaim WD, Longenecker G, Quirk JM, Cardarelli CO, Sugimoto Y, Pastan I, Gottesman MM, Brady RO, Kulkarni AB. alpha-Galactosidase A deficient mice: a model of Fabry disease. Proceedings of the National Academy of Sciences of the United States of America. 94: 2540-4. PMID 9122231 DOI: 10.1073/Pnas.94.6.2540  0.31
1996 BRADY RO, KANFER JN, SHAPIRO D. METABOLISM OF GLUCOCEREBROSIDES. II. EVIDENCE OF AN ENZYMATIC DEFICIENCY IN GAUCHER'S DISEASE. Biochemical and Biophysical Research Communications. 18: 221-5. PMID 14282020 DOI: 10.1016/0006-291X(65)90743-6  0.303
1996 SOMMERS JE, KANFER JN, BRADY RO. SPHINGOLIPID ANTIBODIES. II. SPECIFICITY OF ANTIBODY IN PATIENTS WITH DEMYELINATING DISEASES. Proceedings of the Society For Experimental Biology and Medicine. Society For Experimental Biology and Medicine (New York, N.Y.). 114: 350-2. PMID 14101186 DOI: 10.3181/00379727-114-28673  0.315
1996 Hill SC, Damaska BM, Tsokos M, Kreps C, Brady RO, Barton NW. Radiographic findings in type 3b Gaucher disease. Pediatric Radiology. 26: 852-60. PMID 8929296 DOI: 10.1007/Bf03178036  0.758
1996 Dunbar PIC, Kohn D, Karlsson SCS, Barton IN, Brady R, Cottler-Fox M, Emmons R, Esplin J, Leitman S, Lenarsky C, Nolta J, Parkman R, Pensiero M, Schifmann R, Tolstoshev P, et al. Retroviral Mediated Transfer of the cDNA for Human Glucocerebrosidase into Hematopoietic Stem Cells of Patients with Gaucher Disease. A Phase I Study. National Institutes of Health, Bethesda, Maryland Human Gene Therapy. 7: 231-253. PMID 8788174 DOI: 10.1089/Hum.1996.7.2-231  0.354
1996 Medin JA, Tudor M, Simovitch R, Quirk JM, Jacobson S, Murray GJ, Brady RO. Correction in trans for Fabry disease: expression, secretion and uptake of alpha-galactosidase A in patient-derived cells driven by a high-titer recombinant retroviral vector. Proceedings of the National Academy of Sciences of the United States of America. 93: 7917-22. PMID 8755577 DOI: 10.1073/Pnas.93.15.7917  0.363
1996 Brady RO, Barton NW. Enzyme replacement and gene therapy for Gaucher's disease. Lipids. 31: S137-9. PMID 8729108 DOI: 10.1007/Bf02637065  0.779
1996 Miller SP, Zirzow GC, Doppelt SH, Brady RO, Barton NW. Analysis of the lipids of normal and Gaucher bone marrow. The Journal of Laboratory and Clinical Medicine. 127: 353-8. PMID 8656038 DOI: 10.1016/S0022-2143(96)90183-3  0.746
1996 Medin JA, Migita M, Pawliuk R, Jacobson S, Amiri M, Kluepfel-Stahl S, Brady RO, Humphries RK, Karlsson S. A bicistronic therapeutic retroviral vector enables sorting of transduced CD34+ cells and corrects the enzyme deficiency in cells from Gaucher patients. Blood. 87: 1754-62. PMID 8634421 DOI: 10.1182/Blood.V87.5.1754.Bloodjournal8751754  0.321
1995 Ohshima T, Murray GJ, Nagle JW, Quirk JM, Kraus MH, Barton NW, Brady RO, Kulkarni AB. Structural organization and expression of the mouse gene encoding alpha-galactosidase A. Gene. 166: 277-80. PMID 8543175 DOI: 10.1016/0378-1119(95)00592-7  0.734
1995 Grabowski GA, Barton NW, Pastores G, Dambrosia JM, Banerjee TK, McKee MA, Parker C, Schiffmann R, Hill SC, Brady RO. Enzyme therapy in type 1 Gaucher disease: comparative efficacy of mannose-terminated glucocerebrosidase from natural and recombinant sources. Annals of Internal Medicine. 122: 33-9. PMID 7985893 DOI: 10.7326/0003-4819-122-1-199501010-00005  0.768
1995 Murray GJ, Oliver KL, Jin FS, Brady RO. Studies on the turnover of exogenous mannose-terminal glucocerebrosidase in rat liver lysosomes. Journal of Cellular Biochemistry. 57: 208-17. PMID 7759558 DOI: 10.1002/Jcb.240570205  0.338
1995 Zimran A, Elstein D, Schiffmann R, Abrahamov A, Goldberg M, Bar-Maor JA, Brady RO, Guzzetta PC, Barton NW. Outcome of partial splenectomy for type I Gaucher disease. The Journal of Pediatrics. 126: 596-7. PMID 7699540 DOI: 10.1016/S0022-3476(95)70358-6  0.777
1995 Tedeschi G, Schiffmann R, Barton NW, Shih HH, Gospe SM, Brady RO, Alger JR, Di Chiro G. Proton magnetic resonance spectroscopic imaging in childhood ataxia with diffuse central nervous system hypomyelination. Neurology. 45: 1526-32. PMID 7644053 DOI: 10.1212/Wnl.45.8.1526  0.746
1994 Brady RO, Murray GJ, Barton NW. Modifying exogenous glucocerebrosidase for effective replacement therapy in Gaucher disease. Journal of Inherited Metabolic Disease. 17: 510-9. PMID 7967500 DOI: 10.1007/Bf00711365  0.389
1994 Higgins JJ, Kaneski CR, Bernardini I, Brady RO, Barton NW. Pyridoxine-responsive hyper-beta-alaninemia associated with Cohen's syndrome. Neurology. 44: 1728-32. PMID 7936305 DOI: 10.1212/Wnl.44.9.1728  0.76
1994 Goldin E, Imai Y, Kaneski CR, Pentchev PG, Brady RO, Hascall VC. Mucolipidosis IV fibroblasts synthesize normal amounts of hyaluronic acid. Journal of Inherited Metabolic Disease. 17: 545-53. PMID 7837760 DOI: 10.1007/Bf00711589  0.315
1993 Smanik EJ, Tavill AS, Jacobs GH, Schafer IA, Farquhar L, Weber FL, Mayes JT, Schulak JA, Petrelli M, Zirzow GC, Oliver KL, Miller SPF, Brady RO. Orthotopic liver transplantation in two adults with Niemann-Pick and Gaucher's diseases: Implications for the treatment of inherited metabolic disease Hepatology. 17: 42-49. PMID 8423040 DOI: 10.1002/Hep.1840170109  0.351
1993 Hill SC, Parker CC, Brady RO, Barton NW. MRI of multiple platyspondyly in Gaucher disease: response to enzyme replacement therapy. Journal of Computer Assisted Tomography. 17: 806-9. PMID 8370839 DOI: 10.1097/00004728-199309000-00026  0.764
1993 Banerjee TK, Grubb W, Otero C, McKee M, Brady RO, Barton NW. Musculocutaneous mononeuropathy complicating Capnocytophaga canimorsus infection. Neurology. 43: 2411-2. PMID 8232970 DOI: 10.1212/Wnl.43.11.2411  0.711
1993 Brady RO, Barton NW, Grabowski GA. The role of neurogenetics in Gaucher disease. Archives of Neurology. 50: 1212-24. PMID 8215980 DOI: 10.1001/Archneur.1993.00540110088009  0.784
1992 Barton NW, Brady RO, Dambrosia JM, Doppelt SH, Hill SC, Holder CA, Mankin HJ, Murray GJ, Zirzow GC, Parker RI. Dose-dependent responses to macrophage-targeted glucocerebrosidase in a child with Gaucher disease. The Journal of Pediatrics. 120: 277-80. PMID 1735829 DOI: 10.1016/S0022-3476(05)80444-5  0.739
1992 Higgins JJ, Patterson MC, Papadopoulos NM, Brady RO, Pentchev PG, Barton NW. Hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP syndrome). Neurology. 42: 194-8. PMID 1734303 DOI: 10.1212/Wnl.42.1.194  0.766
1992 Johnson LA, Hoppel BE, Gerard EL, Miller SP, Doppelt SH, Zirzow GC, Rosenthal DI, Dambrosia JM, Hill SC, Brady RO. Quantitative chemical shift imaging of vertebral bone marrow in patients with Gaucher disease. Radiology. 182: 451-5. PMID 1732964 DOI: 10.1148/Radiology.182.2.1732964  0.309
1992 Hill SC, Damaska BM, Ling A, Patterson K, Di Bisceglie AM, Brady RO, Barton NW. Gaucher disease: abdominal MR imaging findings in 46 patients. Radiology. 184: 561-6. PMID 1620865 DOI: 10.1148/Radiology.184.2.1620865  0.757
1992 Filling-Katz MR, Miller SPF, Merrick HF, Travis WD, Gregg RE, Tsokos M, Comly M, Kaneski CR, Mackie S, Lebovics RS, Blanchette-Mackie EJ, Brady RO, Pentchev PG. Clinical, pathologic, and biochemical features of a cholesterol lipidosis accompanied by hyperlipidemia and xanthomas Neurology. 42: 1768-1774. PMID 1513468 DOI: 10.1212/Wnl.42.9.1768  0.33
1992 Higgins JJ, Patterson MC, Dambrosia JM, Pikus AT, Pentchev PG, Sato S, Brady RO, Barton NW. A clinical staging classification for type C Niemann-Pick disease. Neurology. 42: 2286-90. PMID 1461380 DOI: 10.1212/Wnl.42.12.2286  0.761
1992 Rosenthal DI, Barton NW, McKusick KA, Rosen BR, Hill SC, Castronovo FP, Brady RO, Doppelt SH, Mankin HJ. Quantitative imaging of Gaucher disease. Radiology. 185: 841-5. PMID 1438773 DOI: 10.1148/Radiology.185.3.1438773  0.754
1992 Goldin E, Roff CF, Miller SP, Rodriguez-Lafrasse C, Vanier MT, Brady RO, Pentchev PG. Type C Niemann-Pick disease: a murine model of the lysosomal cholesterol lipidosis accumulates sphingosine and sphinganine in liver. Biochimica Et Biophysica Acta. 1127: 303-11. PMID 1324734 DOI: 10.1016/0005-2760(92)90236-O  0.324
1991 Barton NW, Brady RO, Dambrosia JM, Di Bisceglie AM, Doppelt SH, Hill SC, Mankin HJ, Murray GJ, Parker RI, Argoff CE. Replacement therapy for inherited enzyme deficiency--macrophage-targeted glucocerebrosidase for Gaucher's disease. The New England Journal of Medicine. 324: 1464-70. PMID 2023606 DOI: 10.1056/Nejm199105233242104  0.783
1991 Murray GJ, Howard KD, Richards SM, Barton NW, Brady RO. Gaucher's disease: lack of antibody response in 12 patients following repeated intravenous infusions of mannose terminal glucocerebrosidase. Journal of Immunological Methods. 137: 113-20. PMID 2010615 DOI: 10.1016/0022-1759(91)90400-A  0.77
1991 Parker RI, Barton NW, Read EJ, Brady RO. Hematologic improvement in a patient with Gaucher disease on long-term enzyme replacement therapy: evidence for decreased splenic sequestration and improved red blood cell survival. American Journal of Hematology. 38: 130-7. PMID 1951303 DOI: 10.1002/Ajh.2830380211  0.775
1991 Grewal RP, Doppelt SH, Thompson MA, Katz D, Brady RO, Barton NW. Neurologic complications of nonneuronopathic Gaucher's disease. Archives of Neurology. 48: 1271-2. PMID 1845033 DOI: 10.1001/Archneur.1991.00530240075025  0.771
1990 Argoff CE, Kaneski CR, Blanchette-Mackie EJ, Comly M, Dwyer NK, Brown A, Brady RO, Pentchev PG. Type C Niemann-Pick disease: documentation of abnormal LDL processing in lymphocytes. Biochemical and Biophysical Research Communications. 171: 38-45. PMID 2393397 DOI: 10.1016/0006-291X(90)91353-T  0.328
1990 Fink JK, Correll PH, Perry LK, Brady RO, Karlsson S. Correction of glucocerebrosidase deficiency after retroviral-mediated gene transfer into hematopoietic progenitor cells from patients with Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 87: 2334-8. PMID 2315324 DOI: 10.1073/Pnas.87.6.2334  0.315
1990 Barton NW, Furbish FS, Murray GJ, Garfield M, Brady RO. Therapeutic response to intravenous infusions of glucocerebrosidase in a patient with Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 87: 1913-6. PMID 2308952 DOI: 10.1073/Pnas.87.5.1913  0.782
1989 Brady RO, Gal AE, Pentchev PG. Evolution of enzyme replacement therapy for lipid storage diseases. Life Sciences. 15: 1235-48. PMID 4220261 DOI: 10.1016/0024-3205(74)90305-1  0.345
1989 Fink JK, Filling-Katz MR, Sokol J, Cogan DG, Pikus A, Sonies B, Soong B, Pentchev PG, Comly ME, Brady RO. Clinical spectrum of Niemann-Pick disease type C. Neurology. 39: 1040-9. PMID 2761697 DOI: 10.1212/Wnl.39.8.1040  0.338
1989 Brady RO, Filling-Katz MR, Barton NW, Pentchev PG. Niemann-Pick disease types C and D. Neurologic Clinics. 7: 75-88. PMID 2646522 DOI: 10.1016/S0733-8619(18)30829-6  0.763
1989 Fink JK, Ravin P, Argoff CE, Levine RA, Brady RO, Hallett M, Barton NW. Tetrahydrobiopterin administration in biopterin-deficient progressive dystonia with diurnal variation. Neurology. 39: 1393-5. PMID 2477772 DOI: 10.1212/Wnl.39.10.1393  0.744
1988 Vanier MT, Wenger DA, Comly ME, Rousson R, Brady RO, Pentchev PG. Niemann-Pick disease group C: clinical variability and diagnosis based on defective cholesterol esterification. A collaborative study on 70 patients. Clinical Genetics. 33: 331-48. PMID 3378364 DOI: 10.1111/J.1399-0004.1988.Tb03460.X  0.346
1988 Brady RO, Quarles RH. Developmental and pathophysiological aspects of the myelin-associated glycoprotein. Cellular and Molecular Neurobiology. 8: 139-48. PMID 2457442 DOI: 10.1007/Bf00711240  0.309
1986 Cowan MJ, Brady RO, Widder KJ. Elevated erythrocyte adenosine deaminase activity in patients with acquired immunodeficiency syndrome. Proceedings of the National Academy of Sciences of the United States of America. 83: 1089-91. PMID 3006027 DOI: 10.1073/Pnas.83.4.1089  0.312
1985 Murray GJ, Doebber TW, Shen TY, Wu MS, Ponpipom MM, Bugianesi RL, Brady RO, Barranger JA. Targeting of synthetically glycosylated human placental glucocerebrosidase. Biochemical Medicine. 34: 241-6. PMID 4084247 DOI: 10.1016/0006-2944(85)90117-6  0.309
1985 Weintraub H, Abramovici A, Sandbank U, Pentchev PG, Brady RO, Sekine M, Suzuki A, Sela B. Neurological mutation characterized by dysmyelination in NCTR-Balb/C mouse with lysosomal lipid storage disease. Journal of Neurochemistry. 45: 665-72. PMID 4031853 DOI: 10.1111/J.1471-4159.1985.Tb04044.X  0.309
1985 Pentchev PG, Comly ME, Kruth HS, Vanier MT, Wenger DA, Patel S, Brady RO. A defect in cholesterol esterification in Niemann-Pick disease (type C) patients. Proceedings of the National Academy of Sciences of the United States of America. 82: 8247-51. PMID 3865225 DOI: 10.1073/Pnas.82.23.8247  0.304
1985 Ilyas AA, Quarles RH, Dalakas MC, Fishman PH, Brady RO. Monoclonal IgM in a patient with paraproteinemic polyneuropathy binds to gangliosides containing disialosyl groups. Annals of Neurology. 18: 655-9. PMID 2417543 DOI: 10.1002/Ana.410180605  0.31
1985 Yanagisawa K, Quarles RH, Johnson D, Brady RO, Whitaker JN. A derivative of myelin-associated glycoprotein in cerebrospinal fluid of normal subjects and patients with neurological disease. Annals of Neurology. 18: 464-9. PMID 2416268 DOI: 10.1002/Ana.410180409  0.302
1984 Pirruccello S, Barranger JA, Barton NW, Brady RO, Ginns EI. Molecular weight characterization of beta-D-glucocerebrosidase in mononuclear white blood cells in Gaucher's disease. Biochemical Medicine. 31: 73-9. PMID 6743300 DOI: 10.1016/0006-2944(84)90061-9  0.763
1983 Ginns EI, Tegelaers FP, Barneveld R, Galjaard H, Reuser AJ, Brady RO, Tager JM, Barranger JA. Determination of Gaucher's disease phenotypes with monoclonal antibody. Clinica Chimica Acta; International Journal of Clinical Chemistry. 131: 283-7. PMID 6883722 DOI: 10.1016/0009-8981(83)90097-9  0.324
1982 Ginns EI, Brady RO, Pirruccello S, Moore C, Sorrell S, Furbish FS, Murray GJ, Tager J, Barranger JA. Mutations of glucocerebrosidase: discrimination of neurologic and non-neurologic phenotypes of Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 79: 5607-10. PMID 6957882 DOI: 10.1073/Pnas.79.18.5607  0.327
1982 Brady RO. Lysosomal storage diseases. Pharmacology & Therapeutics. 19: 327-36. PMID 6821325 DOI: 10.1016/0163-7258(82)90072-9  0.326
1981 Pentchev PG, Gal AE, Wong R, Morrone S, Neumeyer B, Massey J, Kanter R, Sawitsky A, Brady RO. Biliary excretion of glycolipid in induced or inherited glucosylceramide lipidosis. Biochimica Et Biophysica Acta. 665: 615-8. PMID 7295755 DOI: 10.1016/0005-2760(81)90279-4  0.312
1981 Brady RO, Barranger JA. Therapeutic strategies for lipid storage diseases Trends in Neurosciences. 4: 265-267. DOI: 10.1016/0166-2236(81)90083-7  0.353
1980 Ginns EI, Brady RO, Stowens DW, Furbish FS, Barranger JA. A new group of glucocerebrosidase isozymes found in human white blood cells. Biochemical and Biophysical Research Communications. 97: 1103-7. PMID 7470138 DOI: 10.1016/0006-291X(80)91489-8  0.313
1980 Gal AE, Brady RO, Barranger JA, Pentchev PG. The diagnosis of type A and type B Niemann Pick disease and detection of carriers using leukocytes and a chromogenic analogue of sphingomyelin. Clinica Chimica Acta; International Journal of Clinical Chemistry. 104: 129-32. PMID 7389124 DOI: 10.1016/0009-8981(80)90143-6  0.33
1980 Pentchev PG, Gal AE, Booth AD, Omodeo-Sale F, Fouks J, Neumeyer BA, Quirk JM, Dawson G, Brady RO. A lysosomal storage disorder in mice characterized by a dual deficiency of sphingomyelinase and glucocerebrosidase. Biochimica Et Biophysica Acta. 619: 669-79. PMID 6257302 DOI: 10.1016/0005-2760(80)90116-2  0.321
1980 Rosenberg DM, Ferrans VJ, Fulmer JD, Line BR, Barranger JA, Brady RO, Crystal RG. Chronic airflow obstruction in Fabry's disease. The American Journal of Medicine. 68: 898-905. PMID 6247911 DOI: 10.1016/0002-9343(80)90224-7  0.314
1978 Britton DE, Leinikki PO, Barranger JA, Brady RO. Gaucher's disease: lack of antibody response to intravenous glucocerebrosidase. Life Sciences. 23: 2517-9. PMID 732537 DOI: 10.1016/0024-3205(78)90177-7  0.322
1978 Furbish FS, Steer CJ, Barranger JA, Jones EA, Brady RO. The uptake of native and desialylated glucocerebrosidase by rat hepatocytes and Kupffer cells. Biochemical and Biophysical Research Communications. 81: 1047-53. PMID 666784 DOI: 10.1016/0006-291X(78)91456-0  0.319
1978 Pentchev PG, Kusiak JW, Barranger JA, Furbish FS, Rapoport SI, Massey JM, Brady RO. Factors that influence the uptake and turnover of glucocerebrosidase and alpha-galactosidase in mammalian liver Advances in Experimental Medicine and Biology. 101: 745-752. PMID 208371 DOI: 10.1007/978-1-4615-9071-2_69  0.355
1978 Brady RO. Ophthalmologic aspects of lipid storage diseases. Ophthalmology. 85: 1007-13. PMID 105334 DOI: 10.1016/S0161-6420(78)35592-5  0.364
1978 Pentchev PG, Brady RO, Blair HE, Britton DE, Sorrell SH. Gaucher disease: isolation and comparison of normal and mutant glucocerebrosidase from human spleen tissue. Proceedings of the National Academy of Sciences of the United States of America. 75: 3970-3. PMID 29293 DOI: 10.1073/Pnas.75.8.3970  0.365
1977 Duffard RO, Fishman PH, Bradley RM, Lauter CJ, Brady RO, Trams EG. Ganglioside composition and biosynthesis in cultred cells derived from CNS. Journal of Neurochemistry. 28: 1161-6. PMID 406360 DOI: 10.1111/J.1471-4159.1977.Tb12304.X  0.305
1977 Furbish FS, Blair HE, Shiloach J, Pentchev PG, Brady RO. Enzyme replacement therapy in Gaucher's disease: large-scale purification of glucocerebrosidase suitable for human administration. Proceedings of the National Academy of Sciences of the United States of America. 74: 3560-3. PMID 269414 DOI: 10.1073/Pnas.74.8.3560  0.351
1977 Sakuragawa N, Sakuragaw M, Kuwabara T, Pentchev PG, Barranger JA, Brady RO. Niemann-Pick disease experimental model: sphingomyelinase reduction induced by AY-9944. Science (New York, N.Y.). 196: 317-9. PMID 66749 DOI: 10.1126/Science.66749  0.304
1977 Gal AE, Brady RO, Pentchev PG, Furbish FS, Suzuki K, Tanaka H, Schneider EL. A practical chromogenic procedure for the diagnosis of Krabbe's disease. Clinica Chimica Acta; International Journal of Clinical Chemistry. 77: 53-9. PMID 17489 DOI: 10.1016/0009-8981(77)90401-6  0.365
1977 Brady RO. Heritable catabolic and anabolic disorders of lipid metabolism. Metabolism: Clinical and Experimental. 26: 329-45. PMID 13262 DOI: 10.1016/0026-0495(77)90080-4  0.369
1976 Brady RO, Pentchev PG, Gal AE, Hibbert SR, Quirk JM, Mook GE, Kusiak JW, Tallman JF, Dekaban AS. Enzyme replacement therapy for the sphingolipidoses. Advances in Experimental Medicine and Biology. 68: 523-32. PMID 820173 DOI: 10.1007/978-1-4684-7735-1_34  0.63
1976 Brady RO. Biochemical genetics in neurology. Archives of Neurology. 33: 145-51. PMID 814883 DOI: 10.1001/Archneur.1976.00500030001001  0.356
1975 Tallman JF, Kolodny EH, Brady RO. The preparation of Tay-Sachs ganglioside specifically labeled in either the N-acetylneuraminosyl or N-acetylgalactosaminyl portion of the molecule. Methods in Enzymology. 35: 541-8. PMID 1121293 DOI: 10.1016/0076-6879(75)35182-3  0.55
1975 Fishman PH, Max SR, Tallman JF, Brady RO, Maclaren NK, Cornblath M. Deficient Ganglioside Biosynthesis: a novel human sphingolipidosis. Science (New York, N.Y.). 187: 68-70. PMID 803227 DOI: 10.1126/Science.803227  0.589
1975 Gal AE, Brady RO, Hibbert SR, Pentchev PG. A practical chromogenic procedure for the detection of homozygotes and heterozygous carriers of Niemann-Pick disease. The New England Journal of Medicine. 293: 632-6. PMID 239343 DOI: 10.1056/Nejm197509252931304  0.369
1975 Brady RO. The lipid storage diseases: new concepts and control. Annals of Internal Medicine. 82: 257-61. PMID 163609 DOI: 10.7326/0003-4819-82-2-257  0.375
1974 Tallman JF, Brady RO, Navon R, Padeh B. Ganglioside catabolism in hexosaminidase A-deficient adults. Nature. 252: 254-5. PMID 4421837 DOI: 10.1038/252254A0  0.566
1974 Brady RO, Pentchev PG, Gal AE, Hibbert SR, Dekaban AS. Replacement therapy for inherited enzyme deficiency. Use of purified glucocerebrosidase in Gaucher's disease. The New England Journal of Medicine. 291: 989-93. PMID 4415565 DOI: 10.1056/Nejm197411072911901  0.355
1974 Tallman JF, Brady RO, Quirk JM, Villalba M, Gal AE. Isolation and relationship of human hexosaminidases. The Journal of Biological Chemistry. 249: 3489-99. PMID 4364658  0.466
1974 Tallman JF, Pentchev PG, Brady RO. An enzymological approach to the lipidoses. Enzyme. 18: 136-49. PMID 4211795  0.448
1973 Brady RO, Johnson WG, Uhlendorf BW. Identification of heterozygous carriers of lipid storage diseases. Current status and clinical applications. The American Journal of Medicine. 51: 423-31. PMID 5155766 DOI: 10.1016/0002-9343(71)90249-X  0.366
1973 Tallman JF, Brady RO. The purification and properties of a mammalian neuraminidase (sialidase). Biochimica Et Biophysica Acta. 293: 434-43. PMID 4711815 DOI: 10.1016/0005-2744(73)90350-1  0.461
1973 Schneider EL, Ellis WG, Brady RO, McCulloch JR, Epstein CJ. Infantile (type II) Gaucher's disease: in utero diagnosis and fetal pathology. The Journal of Pediatrics. 81: 1134-9. PMID 4674572 DOI: 10.1016/S0022-3476(72)80245-2  0.327
1973 Brady RO, Tallman JF, Johnson WG, Gal AE, Leahy WR, Quirk JM, Dekaban AS. Replacement therapy for inherited enzyme deficiency. Use of purified ceramidetrihexosidase in Fabry's disease. The New England Journal of Medicine. 289: 9-14. PMID 4196713 DOI: 10.1056/Nejm197307052890103  0.635
1972 Cumar FA, Tallman JF, Brady RO. The biosynthesis of a disialylganglioside by galactosyltransferase from rat brain tissue. The Journal of Biological Chemistry. 247: 2322-7. PMID 5019950  0.479
1972 Tallman JF, Johnson WG, Brady RO. The metabolism of Tay-Sachs ganglioside: catabolic studies with lysosomal enzymes from normal and Tay-Sachs brain tissue. The Journal of Clinical Investigation. 51: 2339-45. PMID 4639018 DOI: 10.1172/Jci107045  0.598
1972 Tallman JF, Brady RO. The catabolism of Tay-Sachs ganglioside in rat brain lysosomes. The Journal of Biological Chemistry. 247: 7570-5. PMID 4636324  0.477
1972 Tallman JF, Brady RO. [109] Neuraminidase (sialidase) from rat heart Methods in Enzymology. 28: 825-829. DOI: 10.1016/0076-6879(72)28115-0  0.513
1972 Quirk JM, Tallman JF, Brady RO. The preparation of trihexosyl - and tetrahexosyl gangliosides specifically labeled in the n-acetylgalactosaminyl moiety Journal of Labelled Compounds. 8: 483-494. DOI: 10.1002/JLCR.2590080314  0.477
1971 Brady RO, Uhlendorf BW, Jacobson CB. Fabry's disease: antenatal detection. Science (New York, N.Y.). 172: 174-5. PMID 5547732 DOI: 10.1126/Science.172.3979.174  0.344
1971 Tallman JF, Brady RO, Suzuki K. Enzymic activities associated with membranous cytoplasmic bodies and isolated brain lysosomes. Journal of Neurochemistry. 18: 1775-7. PMID 4328346  0.507
1970 Austin J, Suzuki K, Armstrong D, Brady R, Bachhawat BK, Schlenker J, Stumpf D. Studies in Globoid (Krabbe) Leukodystrophy (GLD): V. Controlled Enzymic Studies in Ten Human Cases Jama Neurology. 23: 502-512. PMID 5478272 DOI: 10.1001/Archneur.1970.00480300024003  0.312
1970 Brady RO. Prenatal Diagnosis of Lipid Storage Diseases Clinical Chemistry. 16: 811-815. DOI: 10.1093/Clinchem/16.10.811  0.343
1969 Sloan HR, Uhlendorf BW, Kanfer JN, Brady RO, Fredrickson DS. Deficiency of sphingomyelin-cleaving enzyme activity in tissue cultures derived from patients with Niemann-Pick disease. Biochemical and Biophysical Research Communications. 34: 582-8. PMID 5777777 DOI: 10.1016/0006-291X(69)90777-3  0.373
1969 Kolodny EH, Brady RO, Volk BW. Demonstration of an alteration of ganglioside metabolism in Tay-Sachs disease. Biochemical and Biophysical Research Communications. 37: 526-31. PMID 5388728 DOI: 10.1016/0006-291X(69)90947-4  0.348
1969 Brady RO. Tay-sachs disease. The New England Journal of Medicine. 281: 1243-4. PMID 5347842 DOI: 10.1056/Nejm196911272812213  0.366
1969 Snyder RA, Brady RO. The use of white cells as a source of diagnostic material for lipid storage diseases. Clinica Chimica Acta; International Journal of Clinical Chemistry. 25: 331-8. PMID 4895581 DOI: 10.1016/0009-8981(69)90275-7  0.34
1969 Brady RO. Enzymatic defects in the sphingolipidoses. Advances in Clinical Chemistry. 11: 1-19. PMID 4885754 DOI: 10.1016/S0065-2423(08)60055-4  0.34
1969 KATTLOVE HE, WILLIAMS JC, GAYNOR E, SPIVACK M, BRADLEY RM, BRADY RO. Gaucher Cells in Chronic Myelocytic Leukemia: An Acquired Abnormality Blood. 33: 379-390. DOI: 10.1182/Blood.V33.2.379.379  0.319
1967 Brady RO, Gal AE, Bradley RM, Martensson E, Warshaw AL, Laster L. Enzymatic defect in Fabry's disease. Ceramidetrihexosidase deficiency. The New England Journal of Medicine. 276: 1163-7. PMID 6023233 DOI: 10.1056/Nejm196705252762101  0.326
1966 Brady RO, Kanfer JN, Bradley RM, Shapiro D. Demonstration of a deficiency of glucocerebroside-cleaving enzyme in Gaucher's disease. The Journal of Clinical Investigation. 45: 1112-5. PMID 5338605 DOI: 10.1172/Jci105417  0.354
1958 AGRANOFF BW, BRADLEY RM, BRADY RO. The enzymatic synthesis of inositol phosphatide. The Journal of Biological Chemistry. 233: 1077-83. PMID 13598735  0.425
1958 AGRANOFF BW, BRADY RO. Studies with tritium-labeled inositol. Neurology. 8: 79-80. PMID 13541616 DOI: 10.1212/Wnl.8.Suppl_1.79  0.478
1957 AGRANOFF BW, BRADLEY RM, BRADY RO. The role of cytidine nucleotides in the formation of inositol-containing lipid. Biochimica Et Biophysica Acta. 25: 445-6. PMID 13471604 DOI: 10.1016/0006-3002(57)90508-5  0.497
1957 AXELROD J, BRADY RO, WITKOP B, EVARTS EV. The distribution and metabolism of lysergic acid diethylamide. Annals of the New York Academy of Sciences. 66: 435-44. PMID 13425233 DOI: 10.1111/J.1749-6632.1957.Tb40739.X  0.415
1956 AGRANOFF BW, BRADY RO. Purification and properties of calf liver ribokinase. The Journal of Biological Chemistry. 219: 221-9. PMID 13295274  0.424
1954 AGRANOFF BW, BRADY RO, COLODZIN M. Differential conversion of specifically labeled glucose to C14O2. The Journal of Biological Chemistry. 211: 773-9. PMID 13221582  0.41
1953 BRADY RO, WALSER M, AGRANOFF BW. Elaboration of steroid hormones by surviving adrenal tissue slices obtained from thermally stressed dogs. Proceedings of the Society For Experimental Biology and Medicine. Society For Experimental Biology and Medicine (New York, N.Y.). 84: 634-6. PMID 13134240  0.417
1952 BRADY RO, GURIN S. The biosynthesis of fatty acids in cell-free systems. Brookhaven Symposia in Biology. 5: 162-73; discussion, . PMID 13126604  0.547
1952 BRADY RO, GURIN S. Biosynthesis of fatty acids by cell-free or water-soluble enzyme systems. The Journal of Biological Chemistry. 199: 421-31. PMID 12999856  0.562
1951 BRADY RO, LUKENS FD, GURIN S. Synthesis of radioactive fatty acids in vitro and its hormonal control. The Journal of Biological Chemistry. 193: 459-64. PMID 14907734  0.541
1951 BRADY RO, RABINOWITZ J, VAN BAALEN J, GURIN S. The synthesis of radioactive cholesterol and fatty acids in vitro. II. A further study of precursors. The Journal of Biological Chemistry. 193: 137-43. PMID 14907698  0.546
1951 BRADY RO, GURIN S. The biosynthesis of radioactive long-chain fatty acids by homogenized pigeon liver tissue. Archives of Biochemistry and Biophysics. 34: 221-2. PMID 14904055  0.544
1951 BRADY RO, GURIN S. The synthesis of radioactive cholesterol and fatty acids in vitro. The Journal of Biological Chemistry. 189: 371-7. PMID 14832250  0.545
1951 BRADY RO, LUKENS FD, GURIN S. Hormonal influence upon the in vitro synthesis of radioactive fatty acids. Science (New York, N.Y.). 113: 413-5. PMID 14817331 DOI: 10.1126/Science.113.2937.413  0.581
1950 BRADY RO, GURIN S. Biosynthesis of labeled fatty acids and cholesterol in experimental diabetes. The Journal of Biological Chemistry. 187: 589-96. PMID 14803440  0.535
1950 BRADY RO, GURIN S. The biosynthesis of radioactive fatty acids and cholesterol. The Journal of Biological Chemistry. 186: 461-9. PMID 14794641  0.544
1949 CRANDALL DI, BRADY RO, GURIN S. Studies of acetoacetate formation with labeled carbon; the conversion of zeta-(C7)-labeled octanoate to acetoacetate. The Journal of Biological Chemistry. 181: 845-52. PMID 15393802  0.52
Low-probability matches (unlikely to be authored by this person)
2007 Noben-Trauth K, Neely H, Brady RO. Normal hearing in alpha-galactosidase A-deficient mice, the mouse model for Fabry disease. Hearing Research. 234: 10-4. PMID 17933476 DOI: 10.1016/J.Heares.2007.08.009  0.299
1975 Brady RO. The chemistry and control of hereditary lipid diseases. Chemistry and Physics of Lipids. 13: 271-82. PMID 4217661 DOI: 10.1016/0009-3084(74)90003-6  0.299
1985 Das PK, Murray GJ, Zirzow GC, Brady RO, Barranger JA. Lectin-specific targeting of beta-glucocerebrosidase to different liver cells via glycosylated liposomes. Biochemical Medicine. 33: 124-31. PMID 3994697 DOI: 10.1016/0006-2944(85)90135-8  0.298
1967 Kampine JP, Kanfer JN, Gal AE, Bradley RM, Brady RO. Response of sphingolipid hydrolases in spleen and liver to increased erythrocytorhexis. Biochimica Et Biophysica Acta. 137: 135-9. PMID 6030362 DOI: 10.1016/0005-2760(67)90016-1  0.298
1979 Barranger JA, Rapoport SI, Fredericks WR, Pentchev PG, MacDermot KD, Steusing JK, Brady RO. Modification of the blood-brain barrier: increased concentration and fate of enzymes entering the brain. Proceedings of the National Academy of Sciences of the United States of America. 76: 481-5. PMID 284363 DOI: 10.1073/Pnas.76.1.481  0.297
1992 Roff CF, Goldin E, Comly ME, Blanchette-Mackie J, Cooney A, Brady RO, Pentchev PG. Niemann-Pick type-C disease: deficient intracellular transport of exogenously derived cholesterol. American Journal of Medical Genetics. 42: 593-8. PMID 1609841 DOI: 10.1002/Ajmg.1320420433  0.295
1999 Takenaka T, Qin G, Brady RO, Medin JA. Circulating alpha-galactosidase A derived from transduced bone marrow cells: relevance for corrective gene transfer for Fabry disease. Human Gene Therapy. 10: 1931-9. PMID 10466627 DOI: 10.1089/10430349950017293  0.293
1980 Furbish FS, Krett NL, Barranger JA, Brady RO. Fucose plays a role in the clearance and uptake of glucocerebrosidase by rat liver cells. Biochemical and Biophysical Research Communications. 95: 1768-74. PMID 6158326 DOI: 10.1016/S0006-291X(80)80103-3  0.293
1984 Sato S, Quarles RH, Brady RO, Tourtellotte WW. Elevated neutral protease activity in myelin from brains of patients with multiple sclerosis. Annals of Neurology. 15: 264-7. PMID 6202231 DOI: 10.1002/Ana.410150310  0.292
1985 Ilyas AA, Quarles RH, Dalakas MC, Brady RO. Polyneuropathy with monoclonal gammopathy: glycolipids are frequently antigens for IgM paraproteins. Proceedings of the National Academy of Sciences of the United States of America. 82: 6697-700. PMID 2413445 DOI: 10.1073/Pnas.82.19.6697  0.292
2005 Salvioli R, Tatti M, Scarpa S, Moavero SM, Ciaffoni F, Felicetti F, Kaneski CR, Brady RO, Vaccaro AM. The N370S (Asn370-->Ser) mutation affects the capacity of glucosylceramidase to interact with anionic phospholipid-containing membranes and saposin C. The Biochemical Journal. 390: 95-103. PMID 15826241 DOI: 10.1042/Bj20050325  0.291
2005 Kim EY, Hong YB, Lai Z, Cho YH, Brady RO, Jung SC. Long-term expression of the human glucocerebrosidase gene in vivo after transplantation of bone-marrow-derived cells transformed with a lentivirus vector. The Journal of Gene Medicine. 7: 878-87. PMID 15712335 DOI: 10.1002/Jgm.732  0.291
2008 Moore DF, Goldin E, Gelderman MP, Robinson C, Baer J, Ries M, Elkahloun A, Brady RO, Schiffmann R. Apoptotic abnormalities in differential gene expression in peripheral blood mononuclear cells from children with Fabry disease. Acta Paediatrica (Oslo, Norway : 1992). Supplement. 97: 48-52. PMID 18339188 DOI: 10.1111/J.1651-2227.2008.00654.X  0.291
1975 Simmons JL, Fishman PH, Freese E, Brady RO. Morphological alterations and ganglioside sialyltransferase activity induced by small fatty acids in HeLa cells. The Journal of Cell Biology. 66: 414-24. PMID 1141384 DOI: 10.1083/Jcb.66.2.414  0.291
1980 Constantopoulos G, Rees S, Cragg BG, Barranger JA, Brady RO. Experimental animal model for mucopolysaccharidosis: suramin-induced glycosaminoglycan and sphingolipid accumulation in the rat. Proceedings of the National Academy of Sciences of the United States of America. 77: 3700-4. PMID 6774343 DOI: 10.1073/Pnas.77.6.3700  0.291
1986 Rees S, Constantopoulos G, Brady R. The suramin-treated rat as a model of mucopolysaccharidosis: reversibility of biochemical and morphological changes in the liver. Virchows Archiv. B, Cell Pathology Including Molecular Pathology. 51: 235-45. PMID 2874656 DOI: 10.1007/Bf02899033  0.29
1999 Watari H, Blanchette-Mackie EJ, Dwyer NK, Glick JM, Patel S, Neufeld EB, Brady RO, Pentchev PG, Strauss JF. Niemann-Pick C1 protein: obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization. Proceedings of the National Academy of Sciences of the United States of America. 96: 805-10. PMID 9927649 DOI: 10.1073/Pnas.96.3.805  0.29
2007 Lonser RR, Warren KE, Butman JA, Quezado Z, Robison RA, Walbridge S, Schiffman R, Merrill M, Walker ML, Park DM, Croteau D, Brady RO, Oldfield EH. Real-time image-guided direct convective perfusion of intrinsic brainstem lesions. Technical note. Journal of Neurosurgery. 107: 190-7. PMID 17639894 DOI: 10.3171/Jns-07/07/0190  0.29
2016 Dai M, Wang X, Liou B, Inskeep V, Grabowski G, Sun Y, Brady R, Pan D. 521. Platelets Transfusion New Role as Brain Therapeutics for Acute Neuronopathic Gaucher Disease Molecular Therapy. 24: S208. DOI: 10.1016/S1525-0016(16)33330-5  0.29
1974 Max SR, Maclaren NK, Brady RO, Bradley RM, Cornblath M. GM3 GANGLIOSIDOSIS: A NEW LIPID STORAGE DISEASE Pediatric Research. 8: 391-391. DOI: 10.1203/00006450-197404000-00309  0.289
1997 Carstea ED, Morris JA, Coleman KG, Loftus SK, Zhang D, Cummings C, Gu J, Rosenfeld MA, Pavan WJ, Krizman DB, Nagle J, Polymeropoulos MH, Sturley SL, Ioannou YA, Higgins ME, ... ... Brady RO, et al. Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science (New York, N.Y.). 277: 228-31. PMID 9211849 DOI: 10.1126/Science.277.5323.228  0.289
2007 Ries M, Clarke JT, Whybra C, Mehta A, Loveday KS, Brady RO, Beck M, Schiffmann R. Enzyme replacement in Fabry disease: pharmacokinetics and pharmacodynamics of agalsidase alpha in children and adolescents. Journal of Clinical Pharmacology. 47: 1222-30. PMID 17698592 DOI: 10.1177/0091270007305299  0.289
1977 Pentchev PG, Brady RO, Gal AE, Hibbert SR. The isolation and characterization of sphingomyelinase from human placental tissue. Biochimica Et Biophysica Acta. 488: 312-21. PMID 19083 DOI: 10.1016/0005-2760(77)90189-8  0.289
1969 Radin NS, Hof L, Bradley RM, Brady RO. Lactosylceramide galactosidase: comparison with other sphingolipid hydrolases in developing rat brain. Brain Research. 14: 497-505. PMID 5794918 DOI: 10.1016/0006-8993(69)90124-3  0.288
2011 Lu J, Yang C, Chen M, Ye DY, Lonser RR, Brady RO, Zhuang Z. Histone deacetylase inhibitors prevent the degradation and restore the activity of glucocerebrosidase in Gaucher disease. Proceedings of the National Academy of Sciences of the United States of America. 108: 21200-5. PMID 22160715 DOI: 10.1073/Pnas.1119181109  0.287
1994 Brady RO, Barton NW. Enzyme replacement therapy for Gaucher disease: critical investigations beyond demonstration of clinical efficacy. Biochemical Medicine and Metabolic Biology. 52: 1-9. PMID 7917461 DOI: 10.1006/bmmb.1994.1026  0.286
2004 Aizenberg M, Walbridge S, Gary MJ, Vortmeyer AO, Brady RO, Oldfield EH, Lonser RR. 758 Convection-enhanced Delivery of Glucocerebrosidase to the Primate Brain as a Potential Treatment for Neuronopathic Gaucher's Disease Neurosurgery. 55: 471-472. DOI: 10.1227/00006123-200408000-00094  0.286
1984 Ilyas AA, Quarles RH, MacIntosh TD, Dobersen MJ, Trapp BD, Dalakas MC, Brady RO. IgM in a human neuropathy related to paraproteinemia binds to a carbohydrate determinant in the myelin-associated glycoprotein and to a ganglioside. Proceedings of the National Academy of Sciences of the United States of America. 81: 1225-9. PMID 6199795 DOI: 10.1073/Pnas.81.4.1225  0.285
2008 Shin SH, Kluepfel-Stahl S, Cooney AM, Kaneski CR, Quirk JM, Schiffmann R, Brady RO, Murray GJ. Prediction of response of mutated alpha-galactosidase A to a pharmacological chaperone. Pharmacogenetics and Genomics. 18: 773-80. PMID 18698230 DOI: 10.1097/Fpc.0B013E32830500F4  0.284
1983 Barneveld RA, Tegelaers FP, Ginns EI, Visser P, Laanen EA, Brady RO, Galjaard H, Barranger JA, Reuser AJ, Tager JM. Monoclonal antibodies against human beta-glucocerebrosidase. European Journal of Biochemistry / Febs. 134: 585-9. PMID 6192991 DOI: 10.1111/J.1432-1033.1983.Tb07606.X  0.284
2009 Medin J, Walia J, Neschadim A, Fan X, Madden M, Cheung F, Jaffray D, Levade T, Brady R, McCart JA. 87. Outcomes of testing Lentivector-mediated Gene Therapy for Farber Disease in Non-Human Primates Molecular Genetics and Metabolism. 96: S31. DOI: 10.1016/J.Ymgme.2008.11.088  0.284
1986 Gal AE, Weis AL, Quirk JM, Tokoro T, Brady RO. Animal and cellular models of sphingolipid storage disorders of humans. Chemistry and Physics of Lipids. 42: 199-207. PMID 3829206 DOI: 10.1016/0009-3084(86)90053-8  0.283
1976 Brady RO. Inherited metabolic diseases of the nervous system. Science (New York, N.Y.). 193: 733-9. PMID 822514 DOI: 10.1126/Science.822514  0.283
2003 D'Costa J, Harvey-White J, Qasba P, Limaye A, Kaneski CR, Davis-Warren A, Brady RO, Bankiewicz KS, Major EO, Arya SK. HIV-2 derived lentiviral vectors: gene transfer in Parkinson's and Fabry disease models in vitro. Journal of Medical Virology. 71: 173-82. PMID 12938190 DOI: 10.1002/Jmv.10467  0.283
1978 Barranger JA, Pentchev PG, Furbish FS, Steer CJ, Jones EA, Brady RO. Studies of lysosomal function: I. Metabolism of some complex lipids by isolated hepatocytes and Kupffer cells. Biochemical and Biophysical Research Communications. 83: 1055-60. PMID 708424 DOI: 10.1016/0006-291X(78)91502-4  0.283
1973 Brady RO. Hereditary diseases--causes, cures, and problems. Angewandte Chemie (International Ed. in English). 12: 1-11. PMID 4631280 DOI: 10.1002/Anie.197300011  0.283
2008 Gupta SN, Ries M, Murray GJ, Quirk JM, Brady RO, Lidicker JR, Schiffmann R, Moore DF. Skin-impedance in Fabry Disease: a prospective, controlled, non-randomized clinical study. Bmc Neurology. 8: 41. PMID 18990229 DOI: 10.1186/1471-2377-8-41  0.283
2000 Abe A, Gregory S, Lee L, Killen PD, Brady RO, Kulkarni A, Shayman JA. Reduction of globotriaosylceramide in Fabry disease mice by substrate deprivation. The Journal of Clinical Investigation. 105: 1563-71. PMID 10841515 DOI: 10.1172/Jci9711  0.283
1989 Möller JR, Johnson D, Brady RO, Tourtellotte WW, Quarles RH. Antibodies to myelin-associated glycoprotein (MAG) in the cerebrospinal fluid of multiple sclerosis patients. Journal of Neuroimmunology. 22: 55-61. PMID 2465313 DOI: 10.1016/0165-5728(89)90009-X  0.281
1986 Rees S, Constantopoulos G, Brady RO. The suramin-treated rat as a model of mucopolysaccharidosis. Variation in the reversibility of biochemical and morphological changes among different organs. Virchows Archiv. B, Cell Pathology Including Molecular Pathology. 52: 259-72. PMID 2879381 DOI: 10.1007/Bf02889967  0.28
1965 Brady RO, Scow RO, Urgoiti E, Bradley RM. The effect of pancreatectomy on fatty acid synthesis in liver of fasted rats. Biochimica Et Biophysica Acta. 106: 241-7. PMID 5867686 DOI: 10.1016/0005-2760(65)90032-9  0.28
1986 Johnson D, Hafler DA, Fallis RJ, Lees MB, Brady RO, Quarles RH, Weiner HL. Cell-mediated immunity to myelin-associated glycoprotein, proteolipid protein, and myelin basic protein in multiple sclerosis. Journal of Neuroimmunology. 13: 99-108. PMID 2428837 DOI: 10.1016/0165-5728(86)90053-6  0.279
1995 Xu LC, Karlsson S, Byrne ER, Kluepfel-Stahl S, Kessler SW, Agricola BA, Sellers S, Kirby M, Dunbar CE, Brady RO. Long-term in vivo expression of the human glucocerebrosidase gene in nonhuman primates after CD34+ hematopoietic cell transduction with cell-free retroviral vector preparations. Proceedings of the National Academy of Sciences of the United States of America. 92: 4372-6. PMID 7538667 DOI: 10.1073/Pnas.92.10.4372  0.279
2005 Lund N, Branch DR, Sakac D, Lingwood CA, Siatskas C, Robinson CJ, Brady RO, Medin JA. Lack of susceptibility of cells from patients with Fabry disease to productive infection with R5 human immunodeficiency virus. Aids (London, England). 19: 1543-6. PMID 16135910 DOI: 10.1097/01.Aids.0000183521.90878.79  0.278
2001 Itoh Y, Esaki T, Cook M, Qasba P, Shimoji K, Alroy J, Brady RO, Sokoloff L, Moore DF. Local and global cerebral blood flow and glucose utilization in the alpha-galactosidase A knockout mouse model of Fabry disease. Journal of Neurochemistry. 79: 1217-24. PMID 11752062 DOI: 10.1046/J.1471-4159.2001.00669.X  0.277
1973 Pentchev PG, Brady RO. The effect of a heat-stable factor in human spleen on glucocerebrosidase and acid -glucosidase activities. Biochimica Et Biophysica Acta. 297: 491-6. PMID 4705477 DOI: 10.1016/0304-4165(73)90096-2  0.277
1974 Fishman PH, Simmons JL, Brady RO, Freese E. Induction of glycolipid biosynthesis by sodium butyrate in HeLa cells. Biochemical and Biophysical Research Communications. 59: 292-9. PMID 4858378 DOI: 10.1016/S0006-291X(74)80205-6  0.277
1982 Sato S, Quarles RH, Brady RO. Susceptibility of the myelin-associated glycoprotein and basic protein to a neutral protease in highly purified myelin from human and rat brain. Journal of Neurochemistry. 39: 97-105. PMID 6177834 DOI: 10.1111/J.1471-4159.1982.Tb04706.X  0.277
1979 Gal AE, Pentchev PG, Massey JM, Brady RO. L-glucosylceramide: synthesis, properties, and resistance to catabolism by glucocerebrosidase in vitro. Proceedings of the National Academy of Sciences of the United States of America. 76: 3083-6. PMID 290986 DOI: 10.1073/Pnas.76.7.3083  0.277
1972 Mora PT, Cumar FA, Brady RO. A common biochemical change in SV40 and polyoma virus transformed mouse cells coupled to control of cell growth in culture. Virology. 46: 60-72. PMID 4330889 DOI: 10.1016/0042-6822(71)90006-7  0.277
1968 Percy AK, Brady RO. Metachromatic leukodystrophy: diagnosis with samples of venous blood. Science (New York, N.Y.). 161: 594-5. PMID 5668537 DOI: 10.1126/Science.161.3841.594  0.276
1996 Yoshida K, Cleaveland ES, Nagle JW, French S, Yaswen L, Ohshima T, Brady RO, Pentchev PG, Kulkarni AB. Molecular cloning of the mouse apolipoprotein D gene and its upregulated expression in Niemann-Pick disease type C mouse model. Dna and Cell Biology. 15: 873-82. PMID 8892759 DOI: 10.1089/Dna.1996.15.873  0.276
1987 Patel SC, Suresh S, Weintroub H, Brady RO, Pentchev PG. Impaired cholesterol esterification in primary brain cultures of the lysosomal cholesterol storage disorder (LCSD) mouse mutant. Biochemical and Biophysical Research Communications. 143: 233-40. PMID 3827919 DOI: 10.1016/0006-291X(87)90655-3  0.275
1985 Brady RO. Diagnostic and therapeutic applications of sphingolipid hydrolyzing enzymes. Current Topics in Cellular Regulation. 26: 39-50. PMID 3935381 DOI: 10.1016/B978-0-12-152826-3.50011-5  0.275
1979 Kusiak JW, Toney JH, Quirk JM, Brady RO. Specific binding of 125I-labeled beta-hexosaminidase A to rat brain synaptosomes. Proceedings of the National Academy of Sciences of the United States of America. 76: 982-5. PMID 85305 DOI: 10.1073/Pnas.76.2.982  0.275
1985 Dobersen MJ, Gascon P, Trost S, Hammer JA, Goodman S, Noronha AB, O'Shannessy DJ, Brady RO, Quarles RH. Murine monoclonal antibodies to the myelin-associated glycoprotein react with large granular lymphocytes of human blood. Proceedings of the National Academy of Sciences of the United States of America. 82: 552-5. PMID 2578668 DOI: 10.1073/Pnas.82.2.552  0.274
2014 Yang C, Swallows CL, Zhang C, Lu J, Xiao H, Brady RO, Zhuang Z. Celastrol increases glucocerebrosidase activity in Gaucher disease by modulating molecular chaperones. Proceedings of the National Academy of Sciences of the United States of America. 111: 249-54. PMID 24351928 DOI: 10.1073/Pnas.1321341111  0.274
1963 Robinson JD, Bradley RM, Brady RO. The Utilization of Substituted Acyl-Coenzyme A Derivatives in Fatty Acid Synthesis. II. Studies with Enzymes Obtained from Adipose Tissue Biochemistry. 2: 191-194. DOI: 10.1021/Bi00901A035  0.274
2011 Benko W, Ries M, Wiggs EA, Brady RO, Schiffmann R, Fitzgibbon EJ. The saccadic and neurological deficits in type 3 Gaucher disease. Plos One. 6: e22410. PMID 21799847 DOI: 10.1371/Journal.Pone.0022410  0.273
1980 Freese A, Brady RO, Gal AE. A beta-glucosidase in feline kidney that hydrolyzes amygdalin (laetrile). Archives of Biochemistry and Biophysics. 201: 363-8. PMID 6772107 DOI: 10.1016/0003-9861(80)90523-8  0.273
1982 Rees S, Constantopoulos G, Barranger JA, Brady RO. Organomegaly and histopathology in an animal model of mucopolysaccharidosis induced by suramin. Naunyn-Schmiedeberg's Archives of Pharmacology. 319: 262-70. PMID 6810185 DOI: 10.1007/Bf00495876  0.272
1988 Newsom GD, Stanbaugh GH, Kurtzman NA, Brady RO, Gal AE, Vorstad J. Nephrotic syndrome and renal failure associated with a novel glycolipid storage disorder. American Journal of Nephrology. 8: 316-21. PMID 3189427 DOI: 10.1159/000167608  0.272
1986 Willison HJ, Minna JD, Brady RO, Quarles RH. Glycoconjugates in nervous tissue and small cell lung cancer share immunologically cross-reactive carbohydrate determinants. Journal of Neuroimmunology. 10: 353-65. PMID 2418056 DOI: 10.1016/S0165-5728(86)90018-4  0.272
1989 Correll PH, Fink JK, Brady RO, Perry LK, Karlsson S. Production of human glucocerebrosidase in mice after retroviral gene transfer into multipotential hematopoietic progenitor cells. Proceedings of the National Academy of Sciences of the United States of America. 86: 8912-6. PMID 2573069 DOI: 10.1073/Pnas.86.22.8912  0.272
2007 Moore DF, Gelderman MP, Fuhrmann SR, Schiffmann R, Brady RO, Goldin E. Fabry disease and vascular risk factors: future strategies for patient-based studies and the knockout murine model: Fabry disease and vascular risk factors Acta Paediatrica. 95: 69-71. DOI: 10.1111/J.1651-2227.2006.Tb02393.X  0.271
1966 Brady RO, Kanfer JN. [100] Cerebroside glycosidases Methods in Enzymology. 8: 591-597. DOI: 10.1016/0076-6879(66)08106-0  0.271
1973 Quarles RH, Everly JL, Brady RO. Demonstration of a glycoprotein which is associated with a purified myelin fraction from rat brain. Biochemical and Biophysical Research Communications. 47: 491-7. PMID 4350999 DOI: 10.1016/0006-291X(72)90741-3  0.27
1982 Johnson D, Quarles RH, Brady RO. A radioimmunoassay for the myelin-associated glycoprotein. Journal of Neurochemistry. 39: 1356-62. PMID 6181204 DOI: 10.1111/J.1471-4159.1982.Tb12578.X  0.27
1999 Neufeld EB, Wastney M, Patel S, Suresh S, Cooney AM, Dwyer NK, Roff CF, Ohno K, Morris JA, Carstea ED, Incardona JP, Strauss JF, Vanier MT, Patterson MC, Brady RO, et al. The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo. The Journal of Biological Chemistry. 274: 9627-35. PMID 10092649 DOI: 10.1074/Jbc.274.14.9627  0.269
1988 Blanchette-Mackie EJ, Dwyer NK, Amende LM, Kruth HS, Butler JD, Sokol J, Comly ME, Vanier MT, August JT, Brady RO. Type-C Niemann-Pick disease: low density lipoprotein uptake is associated with premature cholesterol accumulation in the Golgi complex and excessive cholesterol storage in lysosomes. Proceedings of the National Academy of Sciences of the United States of America. 85: 8022-6. PMID 3186703 DOI: 10.1073/Pnas.85.21.8022  0.269
1975 Tanaka J, Garcia JH, Max SR, Viloria JE, Kamijyo Y, McLaren NK, Cornblath M, Brady RO. Cerebral sponginess and GM3 gangliosidosis; ultrastructure and probable pathogenesis. Journal of Neuropathology and Experimental Neurology. 34: 249-62. PMID 167132 DOI: 10.1097/00005072-197505000-00003  0.269
1975 Matthieu JM, Quarles RH, Poduslo JF, Brady RO. [35-S]sulfate incorporation into myelin glycoproteinsmi=entral nervous system. Biochimica Et Biophysica Acta. 392: 159-66. PMID 1125324 DOI: 10.1016/0304-4165(75)90176-2  0.269
1986 Johnson D, Sato S, Quarles RH, Inuzuka T, Brady RO, Tourtellotte WW. Quantitation of the myelin-associated glycoprotein in human nervous tissue from controls and multiple sclerosis patients. Journal of Neurochemistry. 46: 1086-93. PMID 2419505 DOI: 10.1111/J.1471-4159.1986.Tb00622.X  0.268
1999 Ohshima T, Gilmore EC, Longenecker G, Jacobowitz DM, Brady RO, Herrup K, Kulkarni AB. Migration defects of cdk5(-/-) neurons in the developing cerebellum is cell autonomous. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 19: 6017-26. PMID 10407039 DOI: 10.1523/Jneurosci.19-14-06017.1999  0.268
2007 Suzuki Y, Ichinomiya S, Kurosawa M, Ohkubo M, Watanabe H, Iwasaki H, Matsuda J, Noguchi Y, Takimoto K, Itoh M, Tabe M, Iida M, Kubo T, Ogawa S, Nanba E, ... ... Brady RO, et al. Chemical chaperone therapy: clinical effect in murine G(M1)-gangliosidosis. Annals of Neurology. 62: 671-5. PMID 17994547 DOI: 10.1002/Ana.21284  0.268
1979 Richards RL, Moss J, Alving CR, Fishman PH, Brady RO. Choleragen (cholera toxin): a bacterial lectin. Proceedings of the National Academy of Sciences of the United States of America. 76: 1673-6. PMID 287006 DOI: 10.1073/Pnas.76.4.1673  0.268
1987 Möller JR, Yanagisawa K, Brady RO, Tourtellotte WW, Quarles RH. Myelin-associated glycoprotein in multiple sclerosis lesions: a quantitative and qualitative analysis. Annals of Neurology. 22: 469-74. PMID 2449119 DOI: 10.1002/Ana.410220405  0.267
1986 Ilyas AA, Dalakas MC, Brady RO, Quarles RH. Sulfated glucuronyl glycolipids reacting with anti-myelin-associated glycoprotein monoclonal antibodies including IgM paraproteins in neuropathy: species distribution and partial characterization of epitopes. Brain Research. 385: 1-9. PMID 2429733 DOI: 10.1016/0006-8993(86)91540-4  0.267
2007 Pareek T, Keller J, Kesavapany S, Utreras E, Agarwal N, Kuner R, Pant H, Iadarola M, Brady R, Kulkarni A. (608) The Journal of Pain. 8: S3. DOI: 10.1016/J.Jpain.2007.02.010  0.266
2001 Weibel TD, Brady RO. Systematic approach to the diagnosis of lysosomal storage disorders. Mental Retardation and Developmental Disabilities Research Reviews. 7: 190-9. PMID 11553935 DOI: 10.1002/Mrdd.1027  0.266
2013 Meng X, Shen J, Brady R, Schiffmann R. In vitro modeling Fabry heart disease using induced pluripotent stem cells Molecular Genetics and Metabolism. 108: S65. DOI: 10.1016/J.Ymgme.2012.11.166  0.266
1984 Neuwelt EA, Barranger JA, Pagel MA, Quirk JM, Brady RO, Frenkel EP. Delivery of active hexosaminidase across the blood-brain barrier in rats. Neurology. 34: 1012-9. PMID 6235460 DOI: 10.1212/Wnl.34.8.1012  0.265
2002 Branton MH, Schiffmann R, Sabnis SG, Murray GJ, Quirk JM, Altarescu G, Goldfarb L, Brady RO, Balow JE, Austin Iii HA, Kopp JB. Natural history of Fabry renal disease: influence of alpha-galactosidase A activity and genetic mutations on clinical course. Medicine. 81: 122-38. PMID 11889412 DOI: 10.1097/00005792-200203000-00003  0.265
1981 Quarles RH, Johnson D, Brady RO, Sternberger NH. Preparation and characterization of antisera to the myelin-associated glycoprotein. Neurochemical Research. 6: 1115-27. PMID 6174880 DOI: 10.1007/Bf00964417  0.265
2010 Darmoise A, Teneberg S, Bouzonville L, Brady RO, Beck M, Kaufmann SH, Winau F. Lysosomal alpha-galactosidase controls the generation of self lipid antigens for natural killer T cells. Immunity. 33: 216-28. PMID 20727792 DOI: 10.1016/J.Immuni.2010.08.003  0.265
1968 Weinreb NJ, Brady RO, Tappel AL. The lysosomal localization of sphingolipid hydrolases. Biochimica Et Biophysica Acta. 159: 141-6. PMID 5650428 DOI: 10.1016/0005-2744(68)90251-9  0.265
1991 Argoff CE, Comly ME, Blanchette-Mackie J, Kruth HS, Pye HT, Goldin E, Kaneski C, Vanier MT, Brady RO, Pentchev PG. Type C Niemann-Pick disease: cellular uncoupling of cholesterol homeostasis is linked to the severity of disruption in the intracellular transport of exogenously derived cholesterol. Biochimica Et Biophysica Acta. 1096: 319-27. PMID 2065103 DOI: 10.1016/0925-4439(91)90068-K  0.264
2000 Rosenberg KM, Schiffmann R, Kaneski C, Brady RO, Sorensen SA, Hasholt L. Five novel mutations in fourteen patients with Fabry Disease. Human Mutation. 15: 207-8. PMID 10649504 DOI: 10.1002/(Sici)1098-1004(200002)15:2<207::Aid-Humu16>3.0.Co;2-C  0.263
1978 McIntyre RJ, Quarles RH, Webster Hd, Brady RO. Isolation And Characterization Of Myelin‐Related Membranes Journal of Neurochemistry. 30: 991-1002. PMID 660201 DOI: 10.1111/J.1471-4159.1978.Tb12391.X  0.263
1995 Sugimoto Y, Aksentijevich I, Murray GJ, Brady RO, Pastan I, Gottesman MM. Retroviral coexpression of a multidrug resistance gene (MDR1) and human alpha-galactosidase A for gene therapy of Fabry disease. Human Gene Therapy. 6: 905-15. PMID 7578409 DOI: 10.1089/Hum.1995.6.7-905  0.262
2015 Xu H, Rahimpour S, Nesvick CL, Zhang X, Ma J, Zhang M, Zhang G, Wang L, Yang C, Hong CS, Germanwala AV, Elder JB, Ray-Chaudhury A, Yao Y, Gilbert MR, ... ... Brady RO, et al. Activation of hypoxia signaling induces phenotypic transformation of glioma cells: implications for bevacizumab antiangiogenic therapy. Oncotarget. 6: 11882-93. PMID 25957416 DOI: 10.18632/Oncotarget.3592  0.262
1976 Moss J, Fishman PH, Manganiello VC, Vaughan M, Brady RO. Functional incorporation of ganglioside into intact cells: induction of choleragen responsiveness. Proceedings of the National Academy of Sciences of the United States of America. 73: 1034-7. PMID 177969 DOI: 10.1073/Pnas.73.4.1034  0.262
1975 Matthieu JM, Brady RO, Quarles RH. Change in a myelin-associated glycoprotein in rat brain during development: metabolic aspects. Brain Research. 86: 55-65. PMID 46767 DOI: 10.1016/0006-8993(75)90637-X  0.26
1966 Brady RO. Immunochemical properties of glycolipids. Journal of the American Oil Chemists' Society. 43: 67-9. PMID 5902880 DOI: 10.1007/Bf02641016  0.26
1974 Matthieu JM, Quarles RH, Brady RO, Webster Hde F. Variation of proteins, enzyme markers and gangliosides in myelin subfractions. Biochimica Et Biophysica Acta. 329: 305-17. PMID 4358408 DOI: 10.1016/0304-4165(73)90295-X  0.26
1975 Brady RO. Composition of membranes of cells transformed by tumorigenic DNA and RNA viruses. American Journal of Clinical Pathology. 63: 685-94. PMID 165709 DOI: 10.1093/Ajcp/63.5.685  0.258
1993 Roff CF, Strauss JF, Goldin E, Jaffe H, Patterson MC, Agritellis GC, Hibbs AM, Garfield M, Brady RO, Pentchev PG. The murine Niemann-Pick type C lesion affects testosterone production. Endocrinology. 133: 2913-23. PMID 8243319 DOI: 10.1210/Endo.133.6.8243319  0.258
1974 Quarles RH, Everly JL, Brady RO. Evidence for the close association of a glycoprotein with myelin in rat brain. Journal of Neurochemistry. 21: 1177-91. PMID 4761704 DOI: 10.1111/J.1471-4159.1973.Tb07573.X  0.258
1979 Steer CJ, Kusiak JW, Brady RO, Jones EA. Selective hepatic uptake of human beta-hexosaminidase A by a specific glycoprotein recognition system on sinusoidal cells. Proceedings of the National Academy of Sciences of the United States of America. 76: 2774-8. PMID 288065 DOI: 10.1073/Pnas.76.6.2774  0.258
1974 Matthieu JM, Quarles RH, de Webster HF, Hogan EL, Brady RO. Characterization of the fraction obtained from the CNS of Jimpy mice by a procedure for myelin isolation Journal of Neurochemistry. 23: 517-523. PMID 4370625 DOI: 10.1111/J.1471-4159.1974.Tb06054.X  0.258
1972 Sammeck R, Brady RO. Studies of the catabolism of myelin basic proteins of the rat in situ and in vitro. Brain Research. 42: 441-53. PMID 5050176 DOI: 10.1016/0006-8993(72)90542-2  0.257
1991 Roff CF, Goldin E, Comly ME, Cooney A, Brown A, Vanier MT, Miller SP, Brady RO, Pentchev PG. Type C Niemann-Pick disease: use of hydrophobic amines to study defective cholesterol transport. Developmental Neuroscience. 13: 315-9. PMID 1817037 DOI: 10.1159/000112179  0.257
1989 O'Neill RR, Tokoro T, Kozak CA, Brady RO. Comparison of the chromosomal localization of murine and human glucocerebrosidase genes and of the deduced amino acid sequences. Proceedings of the National Academy of Sciences of the United States of America. 86: 5049-53. PMID 2740343 DOI: 10.1073/Pnas.86.13.5049  0.257
1977 Everly JL, Quarles RH, Brady RO. Proteins and glycoproteins in myelin purified from the developing bovine and human central nervous systems. Journal of Neurochemistry. 28: 95-101. PMID 188988 DOI: 10.1111/J.1471-4159.1977.Tb07713.X  0.256
1998 KANFER JN, BRADY RO. Studies on the structure of gangliosides. I. On the linkage of N-acetyl neuraminic acid in monosialoganglioside. Biochemical and Biophysical Research Communications. 11: 267-9. PMID 13962222 DOI: 10.1016/0006-291X(63)90555-2  0.256
1974 Fishman PH, Brady RO, Bradley RM, Aaronson SA, Todaro GJ. Absence of a specific ganglioside galactosyltransferase in mouse cells transformed by murine sarcoma virus. Proceedings of the National Academy of Sciences of the United States of America. 71: 298-301. PMID 4360937 DOI: 10.1073/Pnas.71.2.298  0.256
1970 Cumar FA, Brady RO, Kolodny EH, McFarland VW, Mora PT. Enzymatic block in the synthesis of gangliosides in DNA virus-transformed tumorigenic mouse cell lines. Proceedings of the National Academy of Sciences of the United States of America. 67: 757-64. PMID 4331720 DOI: 10.1073/Pnas.67.2.757  0.255
1974 Matthieu J, Brady RO, Quarles RH. NEUROLOGY: EVIDENCE FOR THE PARTICIPATION OF A GLICOPROTEIN IN THE EARLY PROCESSES OF MYELINATION Pediatric Research. 8: 134-134. DOI: 10.1203/00006450-197402000-00047  0.255
1974 Matthieu JM, Brady RO, Quarles RH. Developmental change in a myelin-associated glycoprotein: a comparative study in rodents. Developmental Biology. 37: 146-52. PMID 4823498 DOI: 10.1016/0012-1606(74)90175-4  0.255
1983 Barneveld RA, Keijzer W, Tegelaers FP, Ginns EI, Geurts van Kessel A, Brady RO, Barranger JA, Tager JM, Galjaard H, Westerveld A, Reuser AJ. Assignment of the gene coding for human beta-glucocerebrosidase to the region q21-q31 of chromosome 1 using monoclonal antibodies. Human Genetics. 64: 227-31. PMID 6885065 DOI: 10.1007/Bf00279398  0.255
1972 Fishman PH, McFarland VW, Mora PT, Brady RO. Ganglioside biosynthesis in mouse cells: Glycosyltransferase activities in normal and virally-transformed lines Biochemical and Biophysical Research Communications. 48: 48-57. PMID 4339464 DOI: 10.1016/0006-291X(72)90342-7  0.254
1988 Brady RO, O'Neill RR. Therapy for neurological involvement in AIDS. Annals of Neurology. 23: S211-2. PMID 2831800 DOI: 10.1002/Ana.410230749  0.254
2000 Lai Z, Han I, Zirzow G, Brady RO, Reiser J. Intercellular delivery of a herpes simplex virus VP22 fusion protein from cells infected with lentiviral vectors. Proceedings of the National Academy of Sciences of the United States of America. 97: 11297-302. PMID 11027330 DOI: 10.1073/Pnas.97.21.11297  0.253
1967 Brady RO. Enzymatic Abnormalities in Diseases of Sphingolipid Metabolism Clinical Chemistry. 13: 565-577. DOI: 10.1093/CLINCHEM/13.7.565  0.253
1985 Dobersen MJ, Hammer JA, Noronha AB, MacIntosh TD, Trapp BD, Brady RO, Quarles RH. Generation and characterization of mouse monoclonal antibodies to the myelin-associated glycoprotein (MAG). Neurochemical Research. 10: 499-513. PMID 2582290 DOI: 10.1007/Bf00964654  0.253
1976 Mullin BR, Fishman PH, Lee G, Aloj SM, Ledley FD, Winand RJ, Kohn LD, Brady RO. Thyrotropin-ganglioside interactions and their relationship to the structure and function of thyrotropin receptors. Proceedings of the National Academy of Sciences of the United States of America. 73: 842-6. PMID 176657 DOI: 10.1073/Pnas.73.3.842  0.252
1975 Matthieu JM, Everly JL, Brady RO, Quarles RH. [35-S]sulfate incorporation into myelin clycoproteins; II. Peripheral nervous tissue. Biochimica Et Biophysica Acta. 392: 167-74. PMID 1125325 DOI: 10.1016/0304-4165(75)90177-4  0.252
1980 Kusiak JW, Quirk JM, Brady RO. Factors that influence the uptake of beta-hexosaminidase A by rat peritoneal macrophages. Biochemical and Biophysical Research Communications. 94: 199-204. PMID 7387695 DOI: 10.1016/S0006-291X(80)80206-3  0.252
1959 Formica JV, Brady RO. THE ENZYMATIC CARBOXYLATION OF ACETYL COENZYME A Journal of the American Chemical Society. 81: 752-752. DOI: 10.1021/Ja01512A061  0.252
1976 Zimmerman AW, Matthieu JM, Quarles RH, Brady RO, Hsu JM. Hypomyelination in copper-deficient rats. Prenatal and postnatal copper replacement. Archives of Neurology. 33: 111-9. PMID 1252145 DOI: 10.1001/Archneur.1976.00500020039007  0.252
1990 Correll PH, Kew Y, Perry LK, Brady RO, Fink JK, Karlsson S. Expression of human glucocerebrosidase in long-term reconstituted mice following retroviral-mediated gene transfer into hematopoietic stem cells. Human Gene Therapy. 1: 277-87. PMID 2081194 DOI: 10.1089/Hum.1990.1.3-277  0.252
1977 Moss J, Osborne JC, Fishman PH, Brewer HB, Vaughan M, Brady RO. Effect of gangliosides and substrate analogues on the hydrolysis of nicotinamide adenine dinucleotide by choleragen. Proceedings of the National Academy of Sciences of the United States of America. 74: 74-8. PMID 13371 DOI: 10.1073/Pnas.74.1.74  0.251
1974 Matthieu JM, Brady RO, Quarles RH. Anomalies of myelin-associated glycoproteins in 'Quaking' mice. Journal of Neurochemistry. 22: 291-6. PMID 4829954 DOI: 10.1111/J.1471-4159.1974.Tb11592.X  0.251
2000 BURTON RM, SODD MA, BRADY RO. Studies on the biosynthesis of galactolipids. Neurology. 8: 84-5. PMID 13541618 DOI: 10.1212/Wnl.8.Suppl_1.84  0.251
1975 Zimmerman AW, Quarles RH, Webster HdF, Matthieu J, Brady RO. Characterization and protein analysis of myelin subfractions in rat brain: developmental and regional comparisons. Journal of Neurochemistry. 25: 749-757. PMID 1206395 DOI: 10.1111/J.1471-4159.1975.Tb04404.X  0.251
1979 Gal AE, Pentchev PG, Barranger JA, Dambrosia JM, Brady RO. The distribution of glucocerebroside in the liver of patients with Gaucher's disease. Analytical Biochemistry. 95: 127-32. PMID 495946 DOI: 10.1016/0003-2697(79)90195-7  0.25
1976 Fishman PH, Brady RO, Aaronson ST. A comparison of membrane glycoconjugates from mouse cells transformed by murine and primate RNA sarcoma viruses. Biochemistry. 15: 201-8. PMID 174712 DOI: 10.1021/Bi00646A031  0.25
2002 Lai Z, Brady RO. Gene transfer into the central nervous system in vivo using a recombinanat lentivirus vector. Journal of Neuroscience Research. 67: 363-71. PMID 11813241 DOI: 10.1002/Jnr.10137  0.25
1971 Ziboh VA, Brady RO. Inhibition of lipid synthesis in rat skin by tetrolyl-pantetheine. Proceedings of the Society For Experimental Biology and Medicine. Society For Experimental Biology and Medicine (New York, N.Y.). 136: 15-8. PMID 5540599 DOI: 10.3181/00379727-136-35182  0.25
1991 Kaneski CR, Constantopoulos G, Brady RO. Effect of dimethylsulfoxide on the proliferation and glycosaminoglycan synthesis of rat prostate adenocarcinoma cells (PAIII) in vitro: isolation and characterization of DMSO-resistant cells. The Prostate. 18: 47-58. PMID 1987579 DOI: 10.1002/Pros.2990180105  0.25
1974 Druse MJ, Brady RO, Quarles RH. Metabolism of a myelin-associated glycoprotein in developing rat brain. Brain Research. 76: 423-34. PMID 4853181 DOI: 10.1016/0006-8993(74)90819-1  0.25
1998 Mochizuki H, Schwartz JP, Tanaka K, Brady RO, Reiser J. High-titer human immunodeficiency virus type 1-based vector systems for gene delivery into nondividing cells. Journal of Virology. 72: 8873-83. PMID 9765432 DOI: 10.1128/Jvi.72.11.8873-8883.1998  0.25
1995 Rosenthal DI, Doppelt SH, Mankin HJ, Dambrosia JM, Xavier RJ, McKusick KA, Rosen BR, Baker J, Niklason LT, Hill SC, Miller SPF, Brady RO, Barton NW. Enzyme replacement therapy for gaucher disease: Skeletal responses to macrophage-targeted glucocerebrosidase Pediatrics. 96: 629-637. PMID 7567322  0.25
1984 Ilyas AA, Quarles RH, Brady RO. The monoclonal antibody HNK-1 reacts with a human peripheral nerve ganglioside. Biochemical and Biophysical Research Communications. 122: 1206-11. PMID 6477558 DOI: 10.1016/0006-291X(84)91220-8  0.249
1973 Everly JL, Brady RO, Quarles RH. Evidence that the major protein in rat sciatic nerve myelin is a glycoprotein. Journal of Neurochemistry. 21: 329-34. PMID 4724321 DOI: 10.1111/J.1471-4159.1973.Tb04253.X  0.249
2010 Takahashi S, Ohshima T, Hirasawa M, Pareek TK, Bugge TH, Morozov A, Fujieda K, Brady RO, Kulkarni AB. Conditional deletion of neuronal cyclin-dependent kinase 5 in developing forebrain results in microglial activation and neurodegeneration. The American Journal of Pathology. 176: 320-9. PMID 19948833 DOI: 10.2353/Ajpath.2010.081158  0.248
1972 Sammeck R, Martenson RE, Brady RO. Studies of the metabolism of myelin basic proteins in various regions of the central nervous system of immature and adult rats. Brain Research. 34: 241-54. PMID 5172299 DOI: 10.1016/0006-8993(71)90279-4  0.248
2017 Burns RB, Smetana GW, Brady R. Should This Patient Receive an Antidepressant?: Grand Rounds Discussion From Beth Israel Deaconess Medical Center. Annals of Internal Medicine. 167: 192-199. PMID 28761956 DOI: 10.7326/M17-0966  0.248
1987 Matyas GR, Aaronson SA, Brady RO, Fishman PH. Alteration of glycolipids in ras-transfected NIH 3T3 cells. Proceedings of the National Academy of Sciences of the United States of America. 84: 6065-8. PMID 3476926 DOI: 10.1073/Pnas.84.17.6065  0.247
1979 McIntyre LJ, Quarles RH, Brady RO. Lectin-binding proteins in central-nervous-system myelin. Detection of glycoproteins of purified myelin on polyacrylamide gels by [3h]concanavalin A binding. The Biochemical Journal. 183: 205-12. PMID 534493 DOI: 10.1042/Bj1830205  0.247
1970 Brady RO, Mora PT. Alteration in ganglioside pattern and synthesis in SV40-and polyoma virus-transformed mouse cell lines Biochimica Et Biophysica Acta (Bba) - Lipids and Lipid Metabolism. 218: 308-319. DOI: 10.1016/0005-2760(70)90149-9  0.246
1974 Brady RO, Fishman PH. Alterations of complex lipid metabolism in tumorigenic virus transformed cell lines. Advances in Enzyme Regulation. 11: 231-51. PMID 4363199 DOI: 10.1016/0065-2571(73)90018-6  0.245
2012 Yang C, Iyer RR, Yu AC, Yong RL, Park DM, Weil RJ, Ikejiri B, Brady RO, Lonser RR, Zhuang Z. β-Catenin signaling initiates the activation of astrocytes and its dysregulation contributes to the pathogenesis of astrocytomas. Proceedings of the National Academy of Sciences of the United States of America. 109: 6963-8. PMID 22505738 DOI: 10.1073/Pnas.1118754109  0.244
1966 Kanfer JN, Blacklow RS, Warren L, Brady RO. The enzymatic synthesis of gangliosides. I. The incorporation of labeled N-acetylneuraminic acid into monosialoganglioside. Biochemical and Biophysical Research Communications. 14: 287-91. PMID 5836517 DOI: 10.1016/0006-291X(64)90451-6  0.244
2000 Reiser J, Lai Z, Zhang XY, Brady RO. Development of multigene and regulated lentivirus vectors. Journal of Virology. 74: 10589-99. PMID 11044103 DOI: 10.1128/Jvi.74.22.10589-10599.2000  0.244
2013 Lu J, Frerich JM, Turtzo LC, Li S, Chiang J, Yang C, Wang X, Zhang C, Wu C, Sun Z, Niu G, Zhuang Z, Brady RO, Chen X. Histone deacetylase inhibitors are neuroprotective and preserve NGF-mediated cell survival following traumatic brain injury. Proceedings of the National Academy of Sciences of the United States of America. 110: 10747-52. PMID 23754423 DOI: 10.1073/Pnas.1308950110  0.244
2007 Brady R. WITHDRAWN: 77 Current and future therapies for hereditary metabolic disorders Molecular Genetics and Metabolism. DOI: 10.1016/J.Ymgme.2007.08.082  0.243
2007 Brady R. 17 Current and future therapies for hereditary metabolic disorders Molecular Genetics and Metabolism. 92: 15. DOI: 10.1016/J.Ymgme.2007.08.022  0.243
2002 Lai Z, Han I, Park M, Brady RO. Design of an HIV-1 lentiviral-based gene-trap vector to detect developmentally regulated genes in mammalian cells. Proceedings of the National Academy of Sciences of the United States of America. 99: 3651-6. PMID 11891320 DOI: 10.1073/Pnas.062032499  0.242
1984 Inuzuka T, Quarles RH, Noronha AB, Dobersen MJ, Brady RO. A human lymphocyte antigen is shared with a group of glycoproteins in peripheral nerve. Neuroscience Letters. 51: 105-11. PMID 6514226 DOI: 10.1016/0304-3940(84)90270-2  0.242
1973 Brady RO. Erbkrankheiten _ Ursachen, Behandlung und Probleme Angewandte Chemie. 85: 28-38. DOI: 10.1002/Ange.19730850106  0.242
1978 Pacuszka T, Osborne JC, Brady RO, Fishman PH. Interaction of human chorionic gonadotropin with membrane components of rat testes. Proceedings of the National Academy of Sciences of the United States of America. 75: 764-8. PMID 24844 DOI: 10.1073/Pnas.75.2.764  0.241
1996 BRADY RO. STUDIES OF INHIBITORS OF FATTY ACID BIOSYNTHESIS. III. MECHANISM OF ACTION OF TETROLYL-COENZYME A. Biochimica Et Biophysica Acta. 70: 467-8. PMID 14067621 DOI: 10.1016/0006-3002(63)90777-7  0.24
1984 Boothe AD, Weintroub H, Pentchev PG, Jones J, Butler J, Barry JE, Neumeyer B, Stivers JA, Brady RO. A lysosomal storage disorder in the BALB/c mouse: bone marrow transplantation. Veterinary Pathology. 21: 432-41. PMID 6380093 DOI: 10.1177/030098588402100410  0.24
1983 Pentchev PG, Neumeyer B, Svennerholm L, Groth CG, Brady RO. Immunological and catalytic quantitation of splenic glucocerebrosidase from the three clinical forms of Gaucher disease. American Journal of Human Genetics. 35: 621-8. PMID 6881138  0.24
2006 Brady RO. THE ENZYMATIC SYNTHESIS OF FATTY ACIDS BY ALDOL CONDENSATION. Proceedings of the National Academy of Sciences of the United States of America. 44: 993-8. PMID 16590315 DOI: 10.1073/Pnas.44.10.993  0.24
2006 Jiang ZG, Lu XC, Nelson V, Yang X, Pan W, Chen RW, Lebowitz MS, Almassian B, Tortella FC, Brady RO, Ghanbari HA. A multifunctional cytoprotective agent that reduces neurodegeneration after ischemia. Proceedings of the National Academy of Sciences of the United States of America. 103: 1581-6. PMID 16423893 DOI: 10.1073/Pnas.0510573103  0.239
1996 Reiser J, Harmison G, Kluepfel-Stahl S, Brady RO, Karlsson S, Schubert M. Transduction of nondividing cells using pseudotyped defective high-titer HIV type 1 particles. Proceedings of the National Academy of Sciences of the United States of America. 93: 15266-71. PMID 8986799 DOI: 10.1073/Pnas.93.26.15266  0.238
1980 Brady RO, Barranger JA, Gal AE, Pentchev PG, Furbish FS. Status of enzyme replacement therapy for Gaucher disease. Birth Defects Original Article Series. 16: 361-8. PMID 7004519  0.238
1978 Omodeo-Sale F, Brady RO, Fishman PH. Effect of thyroid phospholipids on the interaction of thyrotropin with thyroid membranes. Proceedings of the National Academy of Sciences of the United States of America. 75: 5301-5. PMID 281681 DOI: 10.1073/Pnas.75.11.5301  0.238
1977 Fishman PH, Aloj SM, Kohn LD, Brady RO. Relationship of gangliosides to the structure and function of thyrotropin receptors: their absence on plasma membranes of a thyroid tumor defective in thyrotropin receptor activity. Proceedings of the National Academy of Sciences of the United States of America. 73: 4060-4. PMID 186783 DOI: 10.1073/Pnas.73.11.4060  0.236
1998 Puri A, Hug P, Jernigan K, Barchi J, Kim HY, Hamilton J, Wiels J, Murray GJ, Brady RO, Blumenthal R. The neutral glycosphingolipid globotriaosylceramide promotes fusion mediated by a CD4-dependent CXCR4-utilizing HIV type 1 envelope glycoprotein. Proceedings of the National Academy of Sciences of the United States of America. 95: 14435-40. PMID 9826718 DOI: 10.1073/Pnas.95.24.14435  0.236
2007 Muhs A, Hickman DT, Pihlgren M, Chuard N, Giriens V, Meerschman C, van der Auwera I, van Leuven F, Sugawara M, Weingertner MC, Bechinger B, Greferath R, Kolonko N, Nagel-Steger L, Riesner D, ... Brady RO, et al. Liposomal vaccines with conformation-specific amyloid peptide antigens define immune response and efficacy in APP transgenic mice. Proceedings of the National Academy of Sciences of the United States of America. 104: 9810-5. PMID 17517595 DOI: 10.1073/Pnas.0703137104  0.235
2010 Brady RO. Benefits from unearthing "a biochemical Rosetta Stone". The Journal of Biological Chemistry. 285: 41216-21. PMID 21030589 DOI: 10.1074/Jbc.X110.197954  0.235
1973 Mora PT, Fishman PH, Bassin RH, Brady RO, McFarland VW. Transformation of Swiss 3T3 cells by murine sarcoma virus is followed by decrease in a glycolipid glycosyltransferase Nature New Biol.. 245: 226-229. PMID 4518362 DOI: 10.1038/Newbio245226A0  0.235
1966 Brady RO, Kanfer JN, Mock MB, Fredrickson DS. The metabolism of sphingomyelin. II. Evidence of an enzymatic deficiency in Niemann-Pick diseae. Proceedings of the National Academy of Sciences of the United States of America. 55: 366-9. PMID 5220952 DOI: 10.1073/Pnas.55.2.366  0.234
1997 Lynch TJ, Mattes CE, Singh A, Bradley RM, Brady RO, Dretchen KL. Cocaine detoxification by human plasma butyrylcholinesterase. Toxicology and Applied Pharmacology. 145: 363-71. PMID 9266810 DOI: 10.1006/Taap.1997.8187  0.233
1974 Brady RO, Quarles RH. The enzymology of myelination. Molecular and Cellular Biochemistry. 2: 23-9. PMID 4587537 DOI: 10.1007/Bf01738675  0.233
2004 Goldin E, Stahl S, Cooney AM, Kaneski CR, Gupta S, Brady RO, Ellis JR, Schiffmann R. Transfer of a mitochondrial DNA fragment to MCOLN1 causes an inherited case of mucolipidosis IV. Human Mutation. 24: 460-5. PMID 15523648 DOI: 10.1002/Humu.20094  0.233
1960 Brady RO, Trams EG, Bradley RM. Participation of closely juxtaposed sulfhydryl groups in fatty acid biosynthesis Biochemical and Biophysical Research Communications. 2: 256-258. DOI: 10.1016/0006-291X(60)90178-9  0.232
1996 Ohshima T, Kozak CA, Nagle JW, Pant HC, Brady RO, Kulkarni AB. Molecular cloning and chromosomal mapping of the mouse gene encoding cyclin-dependent kinase 5 regulatory subunit p35. Genomics. 35: 372-5. PMID 8661152 DOI: 10.1006/Geno.1996.0370  0.232
1977 Lee G, Aloj SM, Brady RO, Kohn LD. The structure and function of glycoprotein hormone receptors: ganglioside interactions with human chorionic gonadotropin. Biochemical and Biophysical Research Communications. 73: 370-7. PMID 187196 DOI: 10.1016/0006-291X(76)90717-8  0.232
1996 Ohshima T, Ward JM, Huh CG, Longenecker G, Veeranna, Pant HC, Brady RO, Martin LJ, Kulkarni AB. Targeted disruption of the cyclin-dependent kinase 5 gene results in abnormal corticogenesis, neuronal pathology and perinatal death. Proceedings of the National Academy of Sciences of the United States of America. 93: 11173-8. PMID 8855328 DOI: 10.1073/Pnas.93.20.11173  0.231
1981 Johnson D, Sato S, Quarles RH, deF. Webster H, Brady RO. 28 RADIOIMMUNOASSAY FOR THE MYELIN-ASSOCIATED GLYCOPROTEIN IN MULTIPLE SCLEROSIS BRAINS Journal of Neuropathology and Experimental Neurology. 40: 308. DOI: 10.1097/00005072-198105000-00035  0.23
2006 Pareek TK, Keller J, Kesavapany S, Pant HC, Iadarola MJ, Brady RO, Kulkarni AB. Cyclin-dependent kinase 5 activity regulates pain signaling. Proceedings of the National Academy of Sciences of the United States of America. 103: 791-6. PMID 16407116 DOI: 10.1073/Pnas.0510405103  0.23
1978 Steer CJ, Furbish FS, Barranger JA, Brady RO, Jones EA. The uptake of agalacto-glucocerebrosidase by rat hepatocytes and Kupffer cells. Febs Letters. 91: 202-5. PMID 680122 DOI: 10.1016/0014-5793(78)81172-7  0.228
1971 Max SR, Brady RO. Alteration of the ganglioside composition of skeletal muscle in murine muscular dystrophy. Nature: New Biology. 233: 55-6. PMID 5286359 DOI: 10.1038/Newbio233055A0  0.228
1998 Mattes CE, Belendiuk GW, Lynch TJ, Brady RO, Dretchen KL. Butyrylcholinesterase: An enzyme antidote for cocaine intoxication Addiction Biology. 3: 171-188. DOI: 10.1080/13556219872236  0.227
1979 Macher BA, Pacuszka T, Mullin BR, Sweeley CC, Brady RO, Fishman PH. Isolation and identification of a fucose-containing ganglioside from bovine thyroid gland. Biochimica Et Biophysica Acta. 588: 35-43. PMID 497244 DOI: 10.1016/0304-4165(79)90368-4  0.227
2009 Mistry PK, Weinreb NJ, Brady RO, Grabowski GA. Gaucher disease: resetting the clinical and scientific agenda. American Journal of Hematology. 84: 205-7. PMID 19296473 DOI: 10.1002/Ajh.21384  0.227
1998 Schiffmann R, Dwyer NK, Lubensky IA, Tsokos M, Sutliff VE, Latimer JS, Frei KP, Brady RO, Barton NW, Blanchette-Mackie EJ, Goldin E. Constitutive achlorhydria in mucolipidosis type IV. Proceedings of the National Academy of Sciences of the United States of America. 95: 1207-12. PMID 9448310 DOI: 10.1073/pnas.95.3.1207  0.226
2007 Mullin BR, Pacuszka T, Lee G, Kohn LD, Brady RO, Fishman PH. Thyroid gangliosides with high affinity for thyrotropin: potential role in thyroid regulation. Science (New York, N.Y.). 199: 77-9. PMID 17569488 DOI: 10.1126/Science.199.4324.77  0.226
1970 Brady RO. Metabolic disorders of sphingolipid metabolism in man. Chemistry and Physics of Lipids. 5: 261-9. PMID 5476325 DOI: 10.1016/0009-3084(70)90023-X  0.225
1978 Brady RO, Rosenberg RN. Autosomal dominant neurological disorders. Annals of Neurology. 4: 548-52. PMID 33592 DOI: 10.1002/Ana.410040612  0.224
1978 McIntyre LJ, Quarles RH, Brady RO. Regional studies of myelin-associated glycoprotein in the rat central nervous system. Brain Research. 149: 251-6. PMID 656959 DOI: 10.1016/0006-8993(78)90607-8  0.223
2011 Yang C, Asthagiri AR, Iyer RR, Lu J, Xu DS, Ksendzovsky A, Brady RO, Zhuang Z, Lonser RR. Missense mutations in the NF2 gene result in the quantitative loss of merlin protein and minimally affect protein intrinsic function. Proceedings of the National Academy of Sciences of the United States of America. 108: 4980-5. PMID 21383154 DOI: 10.1073/Pnas.1102198108  0.222
2007 Daniel P, Myers M, Murray G, Schiffmann R, Brady R, Treco D. Comparison of single and multiple doses of agalsidase alfa on tissue accumulation of globotriaosylcermide in Fabry knockout mice Acta Paediatrica. 91: 135-135. DOI: 10.1111/J.1651-2227.2002.Tb03155.X  0.221
1969 Mora PT, Brady RO, Bradley RM, McFarland VW. Gangliosides in DNA virus-transformed and spontaneously transformed tumorigenic mouse cell lines. Proceedings of the National Academy of Sciences of the United States of America. 63: 1290-6. PMID 4311252 DOI: 10.1073/Pnas.63.4.1290  0.22
1996 Van Hove JL, Yang HW, Wu JY, Brady RO, Chen YT. High-level production of recombinant human lysosomal acid alpha-glucosidase in Chinese hamster ovary cells which targets to heart muscle and corrects glycogen accumulation in fibroblasts from patients with Pompe disease. Proceedings of the National Academy of Sciences of the United States of America. 93: 65-70. PMID 8552676  0.22
1982 Brady RO, Barranger JA, Furbish FS, Stowens DW, Ginns EI. Prospects for enzyme replacement therapy in Gaucher disease. Progress in Clinical and Biological Research. 95: 669-80. PMID 7122643  0.22
1969 Kanfer JN, Brady RO. [23] Sphingomyelinase from rat liver (sphingomyelin cholinephosphohydrolase) Methods in Enzymology. 14: 131-133. DOI: 10.1016/S0076-6879(69)14025-2  0.219
1976 Moss J, Fishman PH, Richards RL, Alving CR, Vaughan M, Brady RO. Choleragen-mediated release of trapped glucose from liposomes containing ganglioside GM1. Proceedings of the National Academy of Sciences of the United States of America. 73: 3480-3. PMID 1068459 DOI: 10.1073/Pnas.73.10.3480  0.218
2004 Hirasawa M, Ohshima T, Takahashi S, Longenecker G, Honjo Y, Veeranna, Pant HC, Mikoshiba K, Brady RO, Kulkarni AB. Perinatal abrogation of Cdk5 expression in brain results in neuronal migration defects. Proceedings of the National Academy of Sciences of the United States of America. 101: 6249-54. PMID 15067135 DOI: 10.1073/Pnas.0307322101  0.218
1977 Meldolesi MF, Fishman PH, Aloj SM, Ledley FD, Lee G, Bradley RM, Bradley RM, Brady RO, Kohn LD. Separation of the glycoprotein and ganglioside components of thyrotropin receptor activity in plasma membranes. Biochemical and Biophysical Research Communications. 75: 581-8. PMID 193492 DOI: 10.1016/0006-291X(77)91512-1  0.217
1974 Brady RO. Inborn errors of lipid metabolism. Advances in Enzymology and Related Areas of Molecular Biology. 38: 293-315. PMID 4208446 DOI: 10.1002/9780470122839.Ch6  0.217
1997 Mattes CE, Lynch TJ, Singh A, Bradley RM, Kellaris PA, Brady RO, Dretchen KL. Therapeutic use of butyrylcholinesterase for cocaine intoxication. Toxicology and Applied Pharmacology. 145: 372-80. PMID 9266811 DOI: 10.1006/Taap.1997.8188  0.217
1988 Edelstein RA, Filling-Katz MR, Pentchev P, Gal A, Chandra R, Shawker T, Guzzetta P, Comly M, Kaneski C, Brady RO. Cholesteryl ester storage disease: a patient with massive splenomegaly and splenic abscess. The American Journal of Gastroenterology. 83: 687-92. PMID 3376925  0.215
2001 Tanaka T, Veeranna, Ohshima T, Rajan P, Amin ND, Cho A, Sreenath T, Pant HC, Brady RO, Kulkarni AB. Neuronal cyclin-dependent kinase 5 activity is critical for survival. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 21: 550-8. PMID 11160434 DOI: 10.1523/Jneurosci.21-02-00550.2001  0.214
1978 Furbish FS, Blair HE, Shiloach J, Pentchev PG, Brady RO. Glucocerebrosidase from human placenta. Methods in Enzymology. 50: 529-32. PMID 26844 DOI: 10.1016/0076-6879(78)50058-X  0.213
1978 Kusiak JW, Quirk JM, Brady RO. Ceramide trihexosidase from human placenta. Methods in Enzymology. 50: 533-7. PMID 661600 DOI: 10.1016/0076-6879(72)28121-6  0.213
2003 Schueler UH, Kolter T, Kaneski CR, Blusztajn JK, Herkenham M, Sandhoff K, Brady RO. Toxicity of glucosylsphingosine (glucopsychosine) to cultured neuronal cells: a model system for assessing neuronal damage in Gaucher disease type 2 and 3. Neurobiology of Disease. 14: 595-601. PMID 14678774 DOI: 10.1016/j.nbd.2003.08.016  0.212
1974 Quarles RH, Everly JL, Brady RO. Myelin-associated glycoprotein: a developmental change. Brain Research. 58: 506-9. PMID 4756140 DOI: 10.1016/0006-8993(73)90022-X  0.212
1970 Brady RO. Enzymic defects in lipid-storage diseases. The Biochemical Journal. 117: 8P-10P. PMID 4911752 DOI: 10.1042/BJ1170008P  0.211
1981 Constantopoulos G, Rees S, Cragg BG, Barranger JA, Brady RO. Differential inhibition of beta-hexosaminidase A and beta-hexosaminidase B by suramin. Biochemical and Biophysical Research Communications. 101: 1345-9. PMID 6458294 DOI: 10.1016/0006-291X(81)91595-3  0.211
1974 Brady RO, Fishman PH. Biosynthesis of glycolipids in virus-transformed cells. Biochimica Et Biophysica Acta. 355: 121-48. PMID 4369815 DOI: 10.1016/0304-419X(74)90001-8  0.21
1974 Max SR, Maclaren NK, Brady RO, Bradley RM, Rennels MB, Tanaka J, Garcia JH, Cornblath M. GM3 (hematoside) sphingolipodystrophy. The New England Journal of Medicine. 291: 929-31. PMID 4213132 DOI: 10.1056/Nejm197410312911802  0.21
1976 Mullin BR, Aloj SM, Fishman PH, Lee G, Kohn LD, Brady RO. Cholera toxin interactions with thyrotropin receptors on thyroid plasma membranes. Proceedings of the National Academy of Sciences of the United States of America. 73: 1679-83. PMID 179095 DOI: 10.1073/Pnas.73.5.1679  0.21
1995 Ohshima T, Nagle JW, Pant HC, Joshi JB, Kozak CA, Brady RO, Kulkarni AB. Molecular cloning and chromosomal mapping of the mouse cyclin-dependent kinase 5 gene. Genomics. 28: 585-8. PMID 7490100 DOI: 10.1006/Geno.1995.1194  0.209
1987 Grunnet ML, Patel S, Suresh S, Yamase H, Pentschev P, Brady RO. NEUROPATHOLOGY OF LSCD BALB/C HYPOMYELINATED MOUSE Journal of Neuropathology and Experimental Neurology. 46: 347. DOI: 10.1097/00005072-198705000-00052  0.208
2003 Mariani G, Filocamo M, Giona F, Villa G, Amendola A, Erba P, Buffoni F, Copello F, Pierini A, Minichilli F, Gatti R, Brady RO. Severity of bone marrow involvement in patients with Gaucher's disease evaluated by scintigraphy with 99mTc-sestamibi. Journal of Nuclear Medicine : Official Publication, Society of Nuclear Medicine. 44: 1253-62. PMID 12902415  0.207
1986 Moller JR, Brady RO, Quarles RH. MYELIN-ASSOCIATED GLYCOPROTEIN IN MULTIPLE SCLEROSIS LESIONS Journal of Neuropathology and Experimental Neurology. 45: 356. DOI: 10.1097/00005072-198605000-00126  0.207
2008 Timmons MM, Garbern J, Robey PG, Singleton A, Hernandez DG, Roodman GD, Zaltz I, Mumm S, Wilcox W, Fischbeck KH, Brady RO, Knight MA. WITHDRAWN: Familial Degenerative Encephalopathy with Intracranial Calcification and Metaphyseal Dysplasia. Journal of Bone and Mineral Research : the Official Journal of the American Society For Bone and Mineral Research. PMID 19016590 DOI: 10.1359/Jbmr.081110  0.206
1975 Matthieu JM, Daniel A, Quarles RH, Brady RO. Interactions of concanavalin A and other lectins with CNS myelin. Brain Research. 81: 348-53. PMID 4434202 DOI: 10.1016/0006-8993(74)90951-2  0.205
2001 Li BS, Sun MK, Zhang L, Takahashi S, Ma W, Vinade L, Kulkarni AB, Brady RO, Pant HC. Regulation of NMDA receptors by cyclin-dependent kinase-5. Proceedings of the National Academy of Sciences of the United States of America. 98: 12742-7. PMID 11675505 DOI: 10.1073/Pnas.211428098  0.204
1994 Roff C, Strauss III J, Brady R, Pentchev P. A Contemporary View of Intracellular Cholesterol Movement. Trends in Glycoscience and Glycotechnology. 6: 75-91. DOI: 10.4052/Tigg.6.75  0.204
2007 Pareek TK, Keller J, Kesavapany S, Agarwal N, Kuner R, Pant HC, Iadarola MJ, Brady RO, Kulkarni AB. Cyclin-dependent kinase 5 modulates nociceptive signaling through direct phosphorylation of transient receptor potential vanilloid 1. Proceedings of the National Academy of Sciences of the United States of America. 104: 660-5. PMID 17194758 DOI: 10.1073/Pnas.0609916104  0.204
1972 Dijong I, Mora PT, Brady RO. Gas chromatographic determination of gangliosides in mouse cell lines and in virally transformed derivative lines. Biochemistry. 10: 4039-44. PMID 4334403 DOI: 10.1021/Bi00798A005  0.202
2005 Takahashi S, Ohshima T, Cho A, Sreenath T, Iadarola MJ, Pant HC, Kim Y, Nairn AC, Brady RO, Greengard P, Kulkarni AB. Increased activity of cyclin-dependent kinase 5 leads to attenuation of cocaine-mediated dopamine signaling. Proceedings of the National Academy of Sciences of the United States of America. 102: 1737-42. PMID 15665076 DOI: 10.1073/Pnas.0409456102  0.199
1972 Johnson WG, Mook G, Brady RO. [116] β-Hexosaminidase A from human placenta Methods in Enzymology. 28: 857-861. DOI: 10.1016/0076-6879(72)28122-8  0.198
1997 Schiffmann R, Tedeschi G, Kinkel RP, Trapp BD, Frank JA, Kaneski CR, Brady RO, Barton NW, Nelson L, Yanovski JA. Leukodystrophy in patients with ovarian dysgenesis. Annals of Neurology. 41: 654-61. PMID 9153528 DOI: 10.1002/ana.410410515  0.198
1997 Takcnaka T, Hendrickson CS, Tudor M, Schulmann R, Brady RO, Medin JA. Extending metabolic cooperativity in gene transfer for fabry disease: Transduction of patient bone marrow cells leads to intracellular enzymatic correction and secretion Faseb Journal. 11: A1089.  0.194
1966 Brady RO. The Structure of Lipids By Spectroscopic and X-Ray Techniques. D. Chapman, John Wiley and Sons, Inc., New York, 1965, 323 pp., $10.50 Clinical Chemistry. 12: 320-320. DOI: 10.1093/Clinchem/12.5.320  0.194
2000 SPYROPOULOS CS, BRADY RO. Prolongation of response of node of Ranvier by metal ions. Science (New York, N.Y.). 129: 1366-7. PMID 13658967 DOI: 10.1126/Science.129.3359.1366  0.193
1972 Weinreb NJ, Brady RO. [110] Glucocerebrosidase from bovine spleen (cerebroside-β-glucosidase) Methods in Enzymology. 28: 830-834. DOI: 10.1016/0076-6879(72)28116-2  0.192
2000 Abe A, Arend LJ, Lee L, Lingwood C, Brady RO, Shayman JA. Glycosphingolipid depletion in fabry disease lymphoblasts with potent inhibitors of glucosylceramide synthase. Kidney International. 57: 446-54. PMID 10652021 DOI: 10.1046/j.1523-1755.2000.00864.x  0.192
1986 Quirk JM, Weis AL, Shapiro D, Brady RO. Animal and cellular models of Gaucher's disease. I. Refractoriness of thio analogs of glucocerebroside to enzymatic hydrolysis. Biotechnology and Applied Biochemistry. 8: 96-100. PMID 3828086  0.192
1979 Fishman PH, Moss J, Richards RL, Brady RO, Alving CR. Liposomes as model membranes for ligand-receptor interactions: studies with choleragen and glycolipids. Biochemistry. 18: 2562-7. PMID 444476 DOI: 10.1021/Bi00579A020  0.19
1985 Brady RO. David Shapiro (1903-1985). Chemistry and Physics of Lipids. 38: 315-7. PMID 3910288 DOI: 10.1016/0009-3084(85)90025-8  0.189
1979 Brady RO, Fishman PH. Biotransducers of membrane-mediated information. Advances in Enzymology and Related Areas of Molecular Biology. 50: 303-23. PMID 386721 DOI: 10.1002/9780470122952.Ch6  0.189
1996 Medin J, Migita M, Pawliuk R, Jacobson S, Amiri M, Kluepfel-Stahl S, Brady R, Humphries R, Karlsson S. A bicistronic therapeutic retroviral vector enables sorting of transduced CD34+ cells and corrects the enzyme deficiency in cells from Gaucher patients Blood. 87: 1754-1762. DOI: 10.1182/blood.V87.5.1754.bloodjournal8751754  0.187
2010 Brady RO. Fabry disease - An overview Fabry Disease. xix-xxvii. DOI: 10.1007/978-90-481-9033-1  0.185
1970 Max SR, Nelson PG, Brady RO. The effect of denervation on the composition of muscle gangliosides. Journal of Neurochemistry. 17: 1517-20. PMID 5471912 DOI: 10.1111/J.1471-4159.1970.Tb00519.X  0.182
1969 Brady RO. Genetics and the Sphingolipidoses Medical Clinics of North America. 53: 827-838. DOI: 10.1016/S0025-7125(16)32735-3  0.182
2005 Brady RO, Schiffmann R. Fabry's Disease Peripheral Neuropathy. 2: 1893-1904. DOI: 10.1016/B978-0-7216-9491-7.50084-3  0.177
1981 Gery I, Zigler JS, Brady RO, Barranger JA. Selective effects of glucocerebroside (Gaucher's storage material) on macrophage cultures. The Journal of Clinical Investigation. 68: 1182-9. PMID 6795230 DOI: 10.1172/JCI110363  0.176
1982 Brady RO. The enzymatic defect in Gaucher disease. Progress in Clinical and Biological Research. 95: 309-14. PMID 7122638  0.175
1982 Brady RO. Control and therapy of lipid storage diseases: present status and future strategies. The Alabama Journal of Medical Sciences. 19: 161-4. PMID 7103002  0.17
1978 Schneider EL, Pentchev PG, Hibbert SR, Sawitsky A, Brady RO. A new form of Niemann-Pick disease characterised by temperature-labile sphingomyelinase. Journal of Medical Genetics. 15: 370-4. PMID 216805  0.17
1983 Constantopoulos G, Rees S, Cragg BG, Barranger JA, Brady RO. Suramin-induced storage disease. Mucopolysaccharidosis. The American Journal of Pathology. 113: 266-8. PMID 6416076  0.166
1987 Tokoro T, Gal AE, Gallo LL, Brady RO. Studies of the pathogenesis of Gaucher's disease: tissue distribution and biliary excretion of [14C]L-glucosylceramide in rats. Journal of Lipid Research. 28: 968-72. PMID 3668390  0.165
1996 McCabe ERB, Fine BA, Golbus MS, Greenhouse JB, McGrath GL, New M, O'Brien WE, Rowley PT, Sly WS, Spence MA, Stockman JA, Whyte M, Wilson W, Wolf B, Aerts JMFG, ... ... Brady RO, et al. Gaucher disease: Current issues in diagnosis and treatment Journal of the American Medical Association. 275: 548-553. PMID 8606477 DOI: 10.1001/jama.275.7.548  0.162
1977 Fishman PH, Brady RO. Biosynthesis and function of gangliosides. Science (New York, N.Y.). 194: 906-15. PMID 185697 DOI: 10.1126/SCIENCE.185697  0.162
2009 Rodrigues LG, Ferraz MJ, Rodrigues D, Pais-Vieira M, Lima D, Brady RO, Sousa MM, Sá-Miranda MC. Neurophysiological, behavioral and morphological abnormalities in the Fabry knockout mice. Neurobiology of Disease. 33: 48-56. PMID 18848893 DOI: 10.1016/J.Nbd.2008.09.001  0.16
1982 Brady RO, James SP, Barranger JA. The liver in lipid storage disease: biochemical basis of pathogenesis and clinical features. Progress in Liver Diseases. 7: 331-46. PMID 6810410  0.16
1973 Schneider EL, Ellis WG, Brady RO, McCulloch JR, Epstein CJ. Prenatal Niemann-Pick disease: biochemical and histologic examination of a 19-gestational week fetus. Pediatric Research. 6: 720-9. PMID 4643533 DOI: 10.1203/00006450-197209000-00005  0.157
1972 Schneider EL, Ellis WG, Brady RO, Mcculloch JR, Epstein CJ. Prenatal Niemann-Pick Disease: Biochemical and Histologic Examination of a 19-Gestational Week Fetus Pediatric Research. 6: 720-729. DOI: 10.1203/00006450-197207000-00005  0.157
1973 Brady RO. Hereditary fat-metabolism diseases. Scientific American. 229: 88-97. PMID 4720308 DOI: 10.1038/SCIENTIFICAMERICAN0873-88  0.157
1993 Barton NW, Brady RO, Dambrosia JM. Treatment of Gaucher's disease. The New England Journal of Medicine. 328: 1564-5; author reply. PMID 8479496 DOI: 10.1056/NEJM199305273282112  0.153
2007 Brady RO. The concept of treatment in lysosomal storage diseases Lysosomal Storage Disorders. 37-43. DOI: 10.1007/978-0-387-70909-3_3  0.153
1994 Brady RO, Barton NW. Development of effective enzyme therapy for metabolic storage disorders International Pediatrics. 9: 175-180.  0.151
1983 Ginns EI, Erickson A, Tegelaers FP, Barneveld R, Reuser AJ, Brady RO, Tager JM, Barranger JA. Isozymes of beta-glucosidase: determination of Gaucher's disease phenotypes. Isozymes. 11: 83-93. PMID 6417048  0.151
1981 Neuwelt EA, Barranger JA, Brady RO, Pagel M, Furbish FS, Quirk JM, Mook GE, Frenkel E. Delivery of hexosaminidase A to the cerebrum after osmotic modification of the blood--brain barrier. Proceedings of the National Academy of Sciences of the United States of America. 78: 5838-41. PMID 6946518 DOI: 10.1073/Pnas.78.9.5838  0.143
1999 Patel SC, Suresh S, Kumar U, Hu CY, Cooney A, Blanchette-Mackie EJ, Neufeld EB, Patel RC, Brady RO, Patel YC, Pentchev PG, Ong WY. Localization of Niemann-Pick C1 protein in astrocytes: implications for neuronal degeneration in Niemann- Pick type C disease. Proceedings of the National Academy of Sciences of the United States of America. 96: 1657-62. PMID 9990080 DOI: 10.1073/pnas.96.4.1657  0.143
1990 Filling-Katz M, Fink JK, Oliver KL, Kaneski C, Merrick HF, Argoff CE, Barton N, Brady RO, Gregg RE. Hyperlipidemia as a complication of Niemann-Pick type B disease. Clinical Pediatrics. 29: 670-3. PMID 2265521  0.141
1984 Pentchev PG, Boothe AD, Kruth HS, Weintroub H, Stivers J, Brady RO. A genetic storage disorder in BALB/C mice with a metabolic block in esterification of exogenous cholesterol. The Journal of Biological Chemistry. 259: 5784-91. PMID 6325448  0.14
1996 Medin JA, Tudor M, Simovitch R, Quirk JM, Tacobson S, Murray GJ, Brady RO. Gene therapy for fabry disease: Expression, secretion and uptake of a-galactosidase a (α-gal A) driven by a high titer recombinant retroviral vector Faseb Journal. 10: A1152.  0.136
1988 Sokol J, Blanchette-Mackie J, Kruth HS, Dwyer NK, Amende LM, Butler JD, Robinson E, Patel S, Brady RO, Comly ME. Type C Niemann-Pick disease. Lysosomal accumulation and defective intracellular mobilization of low density lipoprotein cholesterol. The Journal of Biological Chemistry. 263: 3411-7. PMID 3277970  0.133
2015 Schueler U, Kaneski C, Remaley A, Demosky S, Dwyer N, Blanchette-Mackie J, Hanover J, Brady R. A Short Synthetic Peptide Mimetic of Apolipoprotein A1 Mediates Cholesterol and Globotriaosylceramide Efflux from Fabry Fibroblasts. Jimd Reports. PMID 26683465 DOI: 10.1007/8904_2015_507  0.132
1962 Brady RO. THE TOTAL ENZYMIC SYNTHESIS OF CEREBROSIDES Acta Neurologica Scandinavica. 38: 38-39. DOI: 10.1111/j.1600-0404.1962.tb05273.x  0.132
1980 Pentchev PG, Gal AE, Boothe AD, Fouks J, Omodeo-Sale F, Brady RO. A lysosomal storage disorder in mice characterized by the accumulation of several sphingolipids. Birth Defects Original Article Series. 16: 225-30. PMID 7448355  0.127
2019 Baker JT, Dillon DG, Patrick LM, Roffman JL, Brady RO, Pizzagalli DA, Öngür D, Holmes AJ. Functional connectomics of affective and psychotic pathology. Proceedings of the National Academy of Sciences of the United States of America. PMID 30988201 DOI: 10.1073/Pnas.1820780116  0.126
2012 Brady RO, Cooper A, Jensen JE, Tandon N, Cohen B, Renshaw P, Keshavan M, Öngür D. A longitudinal pilot proton MRS investigation of the manic and euthymic states of bipolar disorder. Translational Psychiatry. 2: e160. PMID 22968227 DOI: 10.1038/Tp.2012.84  0.125
1993 Horowitz M, Tzuri G, Eyal N, Berebi A, Kolodny EH, Brady RO, Barton NW, Abrahamov A, Zimran A. Prevalence of nine mutations among Jewish and non-Jewish Gaucher disease patients. American Journal of Human Genetics. 53: 921-30. PMID 8213821  0.122
1983 Rebois RV, Beckner SK, Brady RO, Fishman PH. Mechanism of action of glycopeptide hormones and cholera toxin: what is the role of ADP-ribosylation? Proceedings of the National Academy of Sciences of the United States of America. 80: 1275-9. PMID 6572387  0.122
2012 Brady RO. Foreword Lysosomal Storage Disorders: a Practical Guide. x. DOI: 10.1002/9781118514672  0.12
1995 Goldin E, Blanchette-Mackie EJ, Dwyer NK, Pentchev PG, Brady RO. Cultured skin fibroblasts derived from patients with mucolipidosis 4 are auto-fluorescent. Pediatric Research. 37: 687-92. PMID 7651750 DOI: 10.1203/00006450-199506000-00003  0.118
1956 BRADY RO, STADTMAN ER. Enzymatic thioltransacetylation. Progress in Neurobiology. 1: 26-39. PMID 13359519  0.117
1970 Brady RO, O'Brien JS, Bradley RM, Gal AE. Sphingolipid hydrolases in brain tissue of patients with generalized gangliodosis. Biochimica Et Biophysica Acta. 210: 193-5. PMID 5460531 DOI: 10.1016/0005-2760(70)90079-2  0.117
1999 Wolosker H, Sheth KN, Takahashi M, Mothet JP, Brady RO, Ferris CD, Snyder SH. Purification of serine racemase: biosynthesis of the neuromodulator D-serine. Proceedings of the National Academy of Sciences of the United States of America. 96: 721-5. PMID 9892700 DOI: 10.1073/Pnas.96.2.721  0.116
1981 Constantopoulos G, Rees S, Cragg BG, Barranger JA, Brady RO. Effect of suramin on the activities of degradative enzymes of sphingolipids in rats. Research Communications in Chemical Pathology and Pharmacology. 32: 87-97. PMID 7291729  0.115
1997 Shamburek RD, Pentchev PG, Zech LA, Blanchette-Mackie J, Carstea ED, VandenBroek JM, Cooper PS, Neufeld EB, Phair RD, Brewer HB, Brady RO, Schwartz CC. Intracellular trafficking of the free cholesterol derived from LDL cholesteryl ester is defective in vivo in Niemann-Pick C disease: insights on normal metabolism of HDL and LDL gained from the NP-C mutation. Journal of Lipid Research. 38: 2422-35. PMID 9458266  0.112
1981 Newmark J, Brady RO, Grimley PM, Gal AE, Waller SG, Thistlethwaite JR. Amygdalin (Laetrile) and prunasin beta-glucosidases: distribution in germ-free rat and in human tumor tissue. Proceedings of the National Academy of Sciences of the United States of America. 78: 6513-6. PMID 6796962  0.11
1971 Kolodny EH, Kanfer J, Quirk JM, Brady RO. Properties of a Particle-bound Enzyme from Rat Intestine that Cleaves Sialic Acid from Tay-Sachs Ganglioside Journal of Biological Chemistry. 246: 1426-1431. DOI: 10.1016/s0021-9258(19)76989-0  0.105
1981 Beckner SK, Brady RO, Fishman PH. Reevaluation of the role of gangliosides in the binding and action of thyrotropin. Proceedings of the National Academy of Sciences of the United States of America. 78: 4848-52. PMID 6272278  0.103
1980 Barranger JA, Rapoport SI, Brady RO. Access of enzymes to brain following osmotic alteration of the blood-brain barrier. Birth Defects Original Article Series. 16: 195-205. PMID 6256018  0.101
2020 Chan SY, Brady R, Hwang M, Higgins A, Nielsen K, Öngür D, Hall MH. Heterogeneity of Outcomes and Network Connectivity in Early-Stage Psychosis: A Longitudinal Study. Schizophrenia Bulletin. PMID 32572485 DOI: 10.1093/schbul/sbaa079  0.1
1955 Brady RO. FLUOROACETYL COENZYME A Journal of Biological Chemistry. 217: 213-224. DOI: 10.1016/s0021-9258(19)57172-1  0.098
1974 Yorkston NJ, McHugh RB, Brady R, Serber M, Sergeant HG. Verbal desensitisation in bronchial asthma. Journal of Psychosomatic Research. 18: 371-6. PMID 4154885 DOI: 10.1016/0022-3999(74)90058-0  0.098
1951 Brady R. BIOSYNTHESIS OF RADIOACTIVE TESTOSTERONE IN VITRO Journal of Biological Chemistry. 193: 145-148. DOI: 10.1016/s0021-9258(19)52437-1  0.098
2015 Xu H, Rahimpour S, Nesvick CL, Zhang X, Ma J, Zhang M, Zhang G, Wang L, Yang C, Hong CS, Germanwala AV, Bradley Elder J, Ray-Chaudhury A, Yao Y, Gilbert MR, ... ... Brady RO, et al. Activation of hypoxia signaling induces phenotypic transformation of glioma cells: Implications for bevacizumab antiangiogenic therapy Oncotarget. 6: 11882-11893.  0.094
1957 Brady RO, Koval GJ. BIOSYNTHESIS OF SPHINGOSINE IN VITRO Journal of the American Chemical Society. 79: 2648-2649. DOI: 10.1021/JA01567A079  0.094
1981 Rebois RV, Omedeo-Sale F, Brady RO, Fishman PH. Covalent crosslinking of human chorionic gonadotropin to its receptor in rat testes. Proceedings of the National Academy of Sciences of the United States of America. 78: 2086-9. PMID 6264462  0.094
1992 Roff CF, Pastuszyn A, Strauss JF, Billheimer JT, Vanier MT, Brady RO, Scallen TJ, Pentchev PG. Deficiencies in sex-regulated expression and levels of two hepatic sterol carrier proteins in a murine model of Niemann-Pick type C disease. The Journal of Biological Chemistry. 267: 15902-8. PMID 1639819  0.093
2004 BRADY RO, HEDGES T. Lack of effect of parahydroxypropiophenone in hyperophthalmopathic Graves' disease and diabetes mellitus. The Journal of Clinical Endocrinology and Metabolism. 12: 604-6. PMID 14927712 DOI: 10.1210/JCEM-12-5-604  0.092
2013 Giakoumatos CI, Tandon N, Shah J, Mathew IT, Brady RO, Clementz BA, Pearlson GD, Thaker GK, Tamminga CA, Sweeney JA, Keshavan MS. Are structural brain abnormalities associated with suicidal behavior in patients with psychotic disorders? Journal of Psychiatric Research. 47: 1389-95. PMID 23866739 DOI: 10.1016/J.Jpsychires.2013.06.011  0.092
1983 Brady RO. Genetic errors and enzyme replacement strategies. Research Publications - Association For Research in Nervous and Mental Disease. 60: 181-93. PMID 6130585  0.091
1984 Takasaki S, Murray GJ, Furbish FS, Brady RO, Barranger JA, Kobata A. Structure of the N-asparagine-linked oligosaccharide units of human placental beta-glucocerebrosidase. The Journal of Biological Chemistry. 259: 10112-7. PMID 6469957  0.09
1973 Brady RO. Disorders of lipid metabolism. The Biochemical Journal. 128: 4P-6P. PMID 5085626 DOI: 10.1042/BJ1280004PD  0.084
2021 Low AYT, Goldstein N, Gaunt JR, Huang KP, Zainolabidin N, Yip AKK, Carty JRE, Choi JY, Miller AM, Ho HST, Lenherr C, Baltar N, Azim E, Sessions OM, Ch'ng TH, ... ... Brady RO, et al. Reverse-translational identification of a cerebellar satiation network. Nature. PMID 34789878 DOI: 10.1038/s41586-021-04143-5  0.084
2021 Chan SY, Brady RO, Lewandowski KE, Higgins A, Öngür D, Hall MH. Dynamic and progressive changes in thalamic functional connectivity over the first five years of psychosis. Molecular Psychiatry. PMID 34697450 DOI: 10.1038/s41380-021-01319-3  0.083
2020 Zeng V, Lizano P, Bolo NR, Lutz O, Brady R, Ivleva EI, Dai W, Clementz B, Tamminga C, Pearlson G, Keshavan M. Altered Cerebral Perfusion in Bipolar Disorder: A pCASL MRI Study. Bipolar Disorders. PMID 32583570 DOI: 10.1111/Bdi.12966  0.083
2016 Hager B, Yang AC, Brady R, Meda S, Clementz B, Pearlson GD, Sweeney JA, Tamminga C, Keshavan M. Neural complexity as a potential translational biomarker for psychosis. Journal of Affective Disorders. PMID 27814962 DOI: 10.1016/J.Jad.2016.10.016  0.082
1981 Seil FJ, Quarles RH, Johnson D, Brady RO. Immunization with purified myelin-associated glycoprotein does not evoke myelination-inhibiting or demyelinating antibodies. Brain Research. 209: 470-5. PMID 7225805 DOI: 10.1016/0006-8993(81)90172-4  0.078
1982 Brady RO. Inherited metabolic storage disorders. Annual Review of Neuroscience. 5: 33-56. PMID 6803652 DOI: 10.1146/annurev.ne.05.030182.000341  0.078
2001 Ohshima T, Ogawa M, Veeranna, Hirasawa M, Longenecker G, Ishiguro K, Pant HC, Brady RO, Kulkarni AB, Mikoshiba K. Synergistic contributions of cyclin-dependant kinase 5/p35 and Reelin/Dab1 to the positioning of cortical neurons in the developing mouse brain. Proceedings of the National Academy of Sciences of the United States of America. 98: 2764-9. PMID 11226314 DOI: 10.1073/Pnas.051628498  0.076
1969 Brady RO, Borek C, Bradley RM. Composition and Synthesis of Gangliosides in Rat Hepatocyte and Hepatoma Cell Lines Journal of Biological Chemistry. 244: 6552-6554. DOI: 10.1016/s0021-9258(18)63499-4  0.075
1975 Coleman PL, Fishman PH, Brady RO, Todaro GJ. Altered ganglioside biosynthesis in mouse cell cultures following transformation with chemical carcinogens and x-irradiation. Journal of Biological Chemistry. 250: 55-60. DOI: 10.1016/s0021-9258(19)41978-9  0.074
1982 Doebber TW, Wu MS, Bugianesi RL, Ponpipom MM, Furbish FS, Barranger JA, Brady RO, Shen TY. Enhanced macrophage uptake of synthetically glycosylated human placental beta-glucocerebrosidase. The Journal of Biological Chemistry. 257: 2193-9. PMID 7061417  0.073
1962 Brady RO. Studies on the Total Enzymatic Synthesis of Cerebrosides Journal of Biological Chemistry. 237: PC2416-PC2417. DOI: 10.1016/s0021-9258(19)63457-5  0.073
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