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

295 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any inaccuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2017 Mooney JJ, Brady RO. Lithium + Colchicine: A Potential Strategy to Reduce Pro-inflammatory Effects of Lithium Treatment. Journal of Clinical Psychopharmacology. PMID 29232311 DOI: 10.1097/JCP.0000000000000830  0.32
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  0.96
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.  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
2010 Brady RO. Fabry disease - An overview Fabry Disease. xix-xxvii. DOI: 10.1007/978-90-481-9033-1  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
2008 Moore DF, Brady RO. Systems-biology approach to sphingolipid-storage disorders Future Lipidology. 3: 105-111. DOI: 10.2217/17460875.3.1.105  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
2007 Brady RO. Complex lipid catabolism Lysosomal Storage Disorders. 45-52. DOI: 10.1007/978-0-387-70909-3_4  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
2005 Brady RO, Schiffmann R. Fabry's Disease Peripheral Neuropathy. 2: 1893-1904. DOI: 10.1016/B978-0-7216-9491-7.50084-3  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
2003 Brady RO. Gaucher and Fabry diseases: from understanding pathophysiology to rational therapies. Acta Paediatrica (Oslo, Norway : 1992). Supplement. 92: 19-24. PMID 14989461  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
2002 Schiffmann R, Brady RO. New prospects for the treatment of lysosomal storage diseases. Drugs. 62: 733-42. PMID 11929328  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
2001 Brady RO. Tay-Sachs disease: the search for the enzymatic defect. Advances in Genetics. 44: 51-60. PMID 11596998  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
2001 Brady RO. Commentary Journal of Inherited Metabolic Disease. 24: 11-12. DOI: 10.1023/A:1012495018288  0.96
2000 Brady RO, Schiffmann R. Clinical features of and recent advances in therapy for Fabry disease. Jama. 284: 2771-5. PMID 11105184  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
2000 Mothet JP, Parent AT, Wolosker H, Brady RO, Linden DJ, Ferris CD, Rogawski MA, Snyder SH. D-serine is an endogenous ligand for the glycine site of the N-methyl-D-aspartate receptor. Proceedings of the National Academy of Sciences of the United States of America. 97: 4926-31. PMID 10781100 DOI: 10.1073/pnas.97.9.4926  0.96
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  0.96
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  0.96
2000 Brady RO. Neurogene therapy for the 21st century. Archives of Neurology. 57: 54. PMID 10634437  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
1998 Brady RO. Therapy for the sphingolipidoses. Archives of Neurology. 55: 1055-6. PMID 9708954 DOI: 10.1001/archneur.55.8.1055  0.96
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  0.96
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  0.96
1997 Brady RO. Gaucher's disease: past, present and future. BaillièRe's Clinical Haematology. 10: 621-34. PMID 9497855 DOI: 10.1016/S0950-3536(97)80031-5  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
1997 Schell MJ, Brady RO, Molliver ME, Snyder SH. D-serine as a neuromodulator: regional and developmental localizations in rat brain glia resemble NMDA receptors. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 17: 1604-15. PMID 9030620  0.96
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.  0.96
1997 Ohshima T, Gilmore EC, Herrup K, Brady RO, Kulkarni A. Ontogeny of abnormal brain development in Cdk5(-/-) mice Developmental Brain Dysfunction. 10: 271-281.  0.96
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  0.96
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  0.96
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  0.44
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  0.96
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  0.96
1996 Brady RO, Barton NW. Enzyme replacement and gene therapy for Gaucher's disease. Lipids. 31: S137-9. PMID 8729108  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
1996 Murray GJ, Turpn TH, Camejon TIL, Reinl SJ, Mini SL, Brady RO. Production of recombinant human glucocerebrosidase in plants Faseb Journal. 10: A1126.  0.96
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.  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
1994 Pentchev PG, Brady RO, Blanchette-Mackie EJ, Vanier MT, Carstea ED, Parker CC, Goldin E, Roff CF. The Niemann-Pick C lesion and its relationship to the intracellular distribution and utilization of LDL cholesterol. Biochimica Et Biophysica Acta. 1225: 235-43. PMID 8312368 DOI: 10.1016/0925-4439(94)90001-9  0.96
1994 Schiffmann R, Moller JR, Trapp BD, Shih HH, Farrer RG, Katz DA, Alger JR, Parker CC, Hauer PE, Kaneski CR. Childhood ataxia with diffuse central nervous system hypomyelination. Annals of Neurology. 35: 331-40. PMID 8122885 DOI: 10.1002/ana.410350314  0.96
1994 Brady RO. Potential gene therapy for alcoholism. Exs. 71: 383-93. PMID 8032169  0.96
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  0.96
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  0.96
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  0.96
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  0.96
1994 Brady RO, Barton NW. Development of effective enzyme therapy for metabolic storage disorders International Pediatrics. 9: 175-180.  0.96
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  0.96
1993 Carstea ED, Polymeropoulos MH, Parker CC, Detera-Wadleigh SD, O'Neill RR, Patterson MC, Goldin E, Xiao H, Straub RE, Vanier MT. Linkage of Niemann-Pick disease type C to human chromosome 18. Proceedings of the National Academy of Sciences of the United States of America. 90: 2002-4. PMID 8446622  0.96
1993 Patterson MC, Di Bisceglie AM, Higgins JJ, Abel RB, Schiffmann R, Parker CC, Argoff CE, Grewal RP, Yu K, Pentchev PG. The effect of cholesterol-lowering agents on hepatic and plasma cholesterol in Niemann-Pick disease type C. Neurology. 43: 61-4. PMID 8423912  0.96
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  0.96
1993 Patterson MC, Horowitz M, Abel RB, Currie JN, Yu KT, Kaneski C, Higgins JJ, O'Neill RR, Fedio P, Pikus A. Isolated horizontal supranuclear gaze palsy as a marker of severe systemic involvement in Gaucher's disease. Neurology. 43: 1993-7. PMID 8413956  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.44
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  0.96
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  0.96
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  0.96
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  0.44
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  0.96
1992 Butler JD, Blanchette-Mackie J, Goldin E, O'Neill RR, Carstea G, Roff CF, Patterson MC, Patel S, Comly ME, Cooney A. Progesterone blocks cholesterol translocation from lysosomes. The Journal of Biological Chemistry. 267: 23797-805. PMID 1429719  0.96
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  0.96
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  0.44
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  0.96
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  0.96
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  0.44
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  0.96
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  0.96
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  0.96
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  0.96
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  0.44
1989 Brady RO, Filling-Katz MR, Barton NW, Pentchev PG. Niemann-Pick disease types C and D. Neurologic Clinics. 7: 75-88. PMID 2646522  0.44
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  0.96
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  0.44
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  0.96
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  0.96
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  0.96
1988 Brady RO, O'Neill RR. Therapy for neurological involvement in AIDS. Annals of Neurology. 23: S211-2. PMID 2831800  0.96
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  0.44
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  0.44
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  0.32
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  0.44
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  0.44
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  0.96
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  0.96
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  0.96
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  0.96
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  0.44
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.44
1984 Fishman PH, Bradley RM, Rebois RV, Brady RO. The role of gangliosides in the interaction of human chorionic gonadotropin and cholera toxin with murine Leydig tumor cells. The Journal of Biological Chemistry. 259: 7983-9. PMID 6330091  0.96
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  0.44
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  0.44
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  0.96
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  0.96
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  0.96
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  0.44
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  0.32
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  0.96
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  0.96
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  0.96
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  0.44
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  0.44
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  0.44
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  0.32
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  0.32
1980 Kusiak JW, Quirk JM, Brady RO. Specific binding of beta-hexosaminidase A to rat brain synaptic plasma membranes. Birth Defects Original Article Series. 16: 93-102. PMID 7448364  0.44
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  0.44
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  0.44
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  0.44
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  0.44
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  0.44
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  0.44
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  0.32
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  0.44
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  0.32
1979 Brady RO, Fishman PH. Biotransducers of membrane-mediated information. Advances in Enzymology and Related Areas of Molecular Biology. 50: 303-23. PMID 386721  0.32
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  0.96
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  0.32
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  0.44
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  0.44
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  0.44
1978 Pacuszka T, Duffard RO, Nishimura RN, Brady RO, Fishman PH. Biosynthesis of bovine thyroid gangliosides. The Journal of Biological Chemistry. 253: 5839-46. PMID 670235  0.32
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  0.32
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  0.44
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  0.96
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  0.96
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  0.96
1974 Tallman JF, Brady RO, Navon R, Padeh B. Ganglioside catabolism in hexosaminidase A-deficient adults. Nature. 252: 254-5. PMID 4421837  0.96
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  0.96
1974 Tallman JF, Pentchev PG, Brady RO. An enzymological approach to the lipidoses. Enzyme. 18: 136-49. PMID 4211795  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
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  0.96
1958 AGRANOFF BW, BRADLEY RM, BRADY RO. The enzymatic synthesis of inositol phosphatide. The Journal of Biological Chemistry. 233: 1077-83. PMID 13598735  1
1958 AGRANOFF BW, BRADY RO. Studies with tritium-labeled inositol. Neurology. 8: 79-80. PMID 13541616  1
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  1
1956 AGRANOFF BW, BRADY RO. Purification and properties of calf liver ribokinase. The Journal of Biological Chemistry. 219: 221-9. PMID 13295274  1
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  1
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  1
1952 BRADY RO, GURIN S. The biosynthesis of fatty acids in cell-free systems. Brookhaven Symposia in Biology. 5: 162-73; discussion, . PMID 13126604  1
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  1
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  1
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  1
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  1
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  1
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  1
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  1
1950 BRADY RO, GURIN S. The biosynthesis of radioactive fatty acids and cholesterol. The Journal of Biological Chemistry. 186: 461-9. PMID 14794641  1
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  0.96
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