| Year |
Citation |
Score |
| 2021 |
Walkley SU. Rethinking lysosomes and lysosomal disease. Neuroscience Letters. 762: 136155. PMID 34358625 DOI: 10.1016/j.neulet.2021.136155 |
0.371 |
|
| 2019 |
Davidson J, Molitor E, Moores S, Gale SE, Subramanian K, Jiang X, Sidhu R, Kell P, Zhang J, Fujiwara H, Davidson C, Helquist P, Melancon BJ, Grigalunas M, Liu G, ... ... Walkley SU, et al. 2-Hydroxypropyl-β-cyclodextrin is the active component in a triple combination formulation for treatment of Niemann-Pick C1 disease. Biochimica Et Biophysica Acta. Molecular and Cell Biology of Lipids. PMID 31051283 DOI: 10.1016/J.Bbalip.2019.04.011 |
0.771 |
|
| 2019 |
Davidson C, Gibson A, Gu T, Ali N, Chandler R, Venditti C, Walkley SU, Pavan W. Improved disease amelioration with combination therapy for Niemann-Pick disease type C1 Molecular Genetics and Metabolism. 126: S46. DOI: 10.1016/J.Ymgme.2018.12.100 |
0.756 |
|
| 2018 |
Kerner-Rossi M, Gulinello M, Walkley S, Dobrenis K. Pathobiology of Christianson syndrome: Linking disrupted endosomal-lysosomal function with intellectual disability and sensory impairments. Neurobiology of Learning and Memory. 165: 106867. PMID 29772390 DOI: 10.1016/J.Nlm.2018.05.004 |
0.365 |
|
| 2018 |
Boudewyn LC, Walkley SU. Current concepts in the neuropathogenesis of mucolipidosis type IV. Journal of Neurochemistry. PMID 29770442 DOI: 10.1111/Jnc.14462 |
0.467 |
|
| 2017 |
Ory DS, Ottinger EA, Farhat NY, King KA, Jiang X, Weissfeld L, Berry-Kravis E, Davidson CD, Bianconi S, Keener LA, Rao R, Soldatos A, Sidhu R, Walters KA, Xu X, ... ... Walkley SU, et al. Intrathecal 2-hydroxypropyl-β-cyclodextrin decreases neurological disease progression in Niemann-Pick disease, type C1: a non-randomised, open-label, phase 1-2 trial. Lancet (London, England). PMID 28803710 DOI: 10.1016/S0140-6736(17)31465-4 |
0.761 |
|
| 2017 |
Boudewyn LC, Sikora J, Kuchar L, Ledvinova J, Grishchuk Y, Wang SL, Dobrenis K, Walkley SU. N-butyl-deoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV. Neurobiology of Disease. PMID 28610891 DOI: 10.1016/J.Nbd.2017.06.003 |
0.482 |
|
| 2017 |
Sikora J, Dworski S, Jones EE, Kamani MA, Micsenyi MC, Sawada T, Le Faouder P, Bertrand-Michel J, Dupuy A, Dunn CK, Xuan IC, Casas J, Fabrias G, Hampson DR, Levade T, ... ... Walkley SU, et al. Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities. The American Journal of Pathology. 187: 864-883. PMID 28342444 DOI: 10.1016/J.Ajpath.2016.12.005 |
0.806 |
|
| 2017 |
Yang DS, Stavrides P, Kumar A, Jiang Y, Mohan PS, Ohno M, Dobrenis K, Davidson CD, Saito M, Pawlik M, Huo C, Walkley SU, Nixon RA. Cyclodextrin has conflicting actions on autophagy flux in vivo in brains of normal and Alzheimer model mice. Human Molecular Genetics. PMID 28062666 DOI: 10.1093/Hmg/Ddx001 |
0.757 |
|
| 2016 |
Patterson MC, Walkley SU. Niemann-Pick disease, type C and Roscoe Brady. Molecular Genetics and Metabolism. PMID 27923544 DOI: 10.1016/J.Ymgme.2016.11.008 |
0.496 |
|
| 2016 |
Walkley SU, Davidson CD, Jacoby J, Marella PD, Ottinger EA, Austin CP, Porter FD, Vite CH, Ory DS. Fostering collaborative research for rare genetic disease: the example of niemann-pick type C disease. Orphanet Journal of Rare Diseases. 11: 161. PMID 27903269 DOI: 10.1186/S13023-016-0540-X |
0.743 |
|
| 2016 |
Davidson CD, Fishman YI, Puskás I, Szemán J, Sohajda T, McCauliff LA, Sikora J, Storch J, Vanier MT, Szente L, Walkley SU, Dobrenis K. Efficacy and ototoxicity of different cyclodextrins in Niemann-Pick C disease. Annals of Clinical and Translational Neurology. 3: 366-80. PMID 27231706 DOI: 10.1002/Acn3.306 |
0.772 |
|
| 2016 |
Sikora J, Leddy J, Gulinello M, Walkley SU. X-linked Christianson syndrome: heterozygous female Slc9a6 knockout mice develop mosaic neuropathological changes and related behavioral abnormalities. Disease Models & Mechanisms. 9: 13-23. PMID 26515654 DOI: 10.1242/Dmm.022780 |
0.348 |
|
| 2016 |
Porter FD, Farhat NY, Ottinger EA, McKew JC, Weissfeld L, Machielse B, Berry-Kavis EM, Vite CH, Walkley SU, Ory DS. Phase 1/2 evaluation of intrathecal 2-hydroxypropyl-β-cyclodextrin for the treatment of Niemann-Pick disease type C1 Molecular Genetics and Metabolism. 117: S97. DOI: 10.1016/J.Ymgme.2015.12.411 |
0.368 |
|
| 2016 |
Davidson C, Fishman Y, Puskas I, Szeman J, Sohajda T, Sikora J, Vanier MT, Szente L, Walkley SU, Dobrenis K. Different cyclodextrins for the treatment of Niemann-Pick disease type C Molecular Genetics and Metabolism. 117: S38. DOI: 10.1016/J.Ymgme.2015.12.232 |
0.748 |
|
| 2015 |
Praggastis M, Tortelli B, Zhang J, Fujiwara H, Sidhu R, Chacko A, Chen Z, Chung C, Lieberman AP, Sikora J, Davidson C, Walkley SU, Pipalia NH, Maxfield FR, Schaffer JE, et al. A Murine Niemann-Pick C1 I1061T Knock-In Model Recapitulates the Pathological Features of the Most Prevalent Human Disease Allele. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 35: 8091-106. PMID 26019327 DOI: 10.1523/Jneurosci.4173-14.2015 |
0.764 |
|
| 2015 |
Herbert AS, Davidson C, Kuehne AI, Bakken R, Braigen SZ, Gunn KE, Whelan SP, Brummelkamp TR, Twenhafel NA, Chandran K, Walkley SU, Dye JM. Niemann-pick C1 is essential for ebolavirus replication and pathogenesis in vivo. Mbio. 6: e00565-15. PMID 26015498 DOI: 10.1128/Mbio.00565-15 |
0.731 |
|
| 2015 |
Vite CH, Bagel JH, Swain GP, Prociuk M, Sikora TU, Stein VM, O'Donnell P, Ruane T, Ward S, Crooks A, Li S, Mauldin E, Stellar S, De Meulder M, Kao ML, ... ... Walkley SU, et al. Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease. Science Translational Medicine. 7: 276ra26. PMID 25717099 DOI: 10.1126/Scitranslmed.3010101 |
0.78 |
|
| 2015 |
Kowalewski B, Heimann P, Ortkras T, Lüllmann-Rauch R, Sawada T, Walkley SU, Dierks T, Damme M. Ataxia is the major neuropathological finding in arylsulfatase G-deficient mice: similarities and dissimilarities to Sanfilippo disease (mucopolysaccharidosis type III). Human Molecular Genetics. 24: 1856-68. PMID 25452429 DOI: 10.1093/Hmg/Ddu603 |
0.469 |
|
| 2015 |
Dworski S, Jones EE, Sikora J, Micsenyi M, Sawada T, Le Faouder P, Bertrand-Michel J, Dupuy A, Kamani M, Dunn CK, Xuan I, Hampson DR, Carpentier S, Casas J, Fabrias G, ... ... Walkley SU, et al. Spatial distribution of brain ceramides in an acid ceramidase deficient murine model: Subsequent histological manifestations and functional deficits Molecular Genetics and Metabolism. 114: S38-S39. DOI: 10.1016/J.Ymgme.2014.12.070 |
0.755 |
|
| 2014 |
Yang DS, Stavrides P, Saito M, Kumar A, Rodriguez-Navarro JA, Pawlik M, Huo C, Walkley SU, Saito M, Cuervo AM, Nixon RA. Defective macroautophagic turnover of brain lipids in the TgCRND8 Alzheimer mouse model: prevention by correcting lysosomal proteolytic deficits. Brain : a Journal of Neurology. 137: 3300-18. PMID 25270989 DOI: 10.1093/Brain/Awu278 |
0.448 |
|
| 2014 |
Andrade GN, Molholm S, Butler JS, Brandwein AB, Walkley SU, Foxe JJ. Atypical multisensory integration in Niemann-Pick type C disease - towards potential biomarkers. Orphanet Journal of Rare Diseases. 9: 149. PMID 25239094 DOI: 10.1186/S13023-014-0149-X |
0.361 |
|
| 2014 |
Ottinger EA, Kao ML, Carrillo-Carrasco N, Yanjanin N, Shankar RK, Janssen M, Brewster M, Scott I, Xu X, Cradock J, Terse P, Dehdashti SJ, Marugan J, Zheng W, Portilla L, ... ... Walkley SU, et al. Collaborative development of 2-hydroxypropyl-β-cyclodextrin for the treatment of Niemann-Pick type C1 disease. Current Topics in Medicinal Chemistry. 14: 330-9. PMID 24283970 DOI: 10.2174/1568026613666131127160118 |
0.315 |
|
| 2014 |
Sawada T, Dworski S, Sikora J, Micsenyi M, Medin JA, Walkley SU, Hampson DR. Farber disease: characterization of behavior and brain pathology in a new murine model Molecular Genetics and Metabolism. 111: S93. DOI: 10.1016/J.Ymgme.2013.12.227 |
0.768 |
|
| 2014 |
Davidson C, Jacoby J, Marella P, Recke S, Hadley B, Gallo-Hadley L, Vite C, Walkley SU, Ory DS. SOAR-NPC: A unique collaboration designed to rapidly advance promising therapies to the clinic Molecular Genetics and Metabolism. 111: S34. DOI: 10.1016/J.Ymgme.2013.12.063 |
0.674 |
|
| 2013 |
Fan M, Sidhu R, Fujiwara H, Tortelli B, Zhang J, Davidson C, Walkley SU, Bagel JH, Vite C, Yanjanin NM, Porter FD, Schaffer JE, Ory DS. Identification of Niemann-Pick C1 disease biomarkers through sphingolipid profiling. Journal of Lipid Research. 54: 2800-14. PMID 23881911 DOI: 10.1194/Jlr.M040618 |
0.755 |
|
| 2013 |
Micsenyi MC, Sikora J, Stephney G, Dobrenis K, Walkley SU. Lysosomal membrane permeability stimulates protein aggregate formation in neurons of a lysosomal disease. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 33: 10815-27. PMID 23804102 DOI: 10.1523/Jneurosci.0987-13.2013 |
0.801 |
|
| 2013 |
Pontikis CC, Davidson CD, Walkley SU, Platt FM, Begley DJ. Cyclodextrin alleviates neuronal storage of cholesterol in Niemann-Pick C disease without evidence of detectable blood-brain barrier permeability. Journal of Inherited Metabolic Disease. 36: 491-8. PMID 23412751 DOI: 10.1007/S10545-012-9583-X |
0.751 |
|
| 2013 |
Xu M, Liu K, Swaroop M, Porter FD, Sidhu R, Firnkes S, Ory DS, Marugan JJ, Xiao J, Southall N, Pavan WJ, Davidson C, Walkley SU, Remaley AT, Baxa U, et al. δ-Tocopherol reduces lipid accumulation in Niemann-Pick type C1 and Wolman cholesterol storage disorders (Journal of Biological Chemistry (2012) 287, (39349-39360)) Journal of Biological Chemistry. 288: 296. DOI: 10.1074/Jbc.A112.357707 |
0.72 |
|
| 2012 |
Xu M, Liu K, Swaroop M, Porter FD, Sidhu R, Firnkes S, Finkes S, Ory DS, Marugan JJ, Xiao J, Southall N, Pavan WJ, Davidson C, Walkley SU, Remaley AT, et al. δ-Tocopherol reduces lipid accumulation in Niemann-Pick type C1 and Wolman cholesterol storage disorders. The Journal of Biological Chemistry. 287: 39349-60. PMID 23035117 DOI: 10.1074/Jbc.M112.357707 |
0.79 |
|
| 2012 |
Kollmann K, Damme M, Markmann S, Morelle W, Schweizer M, Hermans-Borgmeyer I, Röchert AK, Pohl S, Lübke T, Michalski JC, Käkelä R, Walkley SU, Braulke T. Lysosomal dysfunction causes neurodegeneration in mucolipidosis II 'knock-in' mice. Brain : a Journal of Neurology. 135: 2661-75. PMID 22961545 DOI: 10.1093/Brain/Aws209 |
0.432 |
|
| 2012 |
Lieberman AP, Puertollano R, Raben N, Slaugenhaupt S, Walkley SU, Ballabio A. Autophagy in lysosomal storage disorders. Autophagy. 8: 719-30. PMID 22647656 DOI: 10.4161/Auto.19469 |
0.357 |
|
| 2012 |
Cluzeau CV, Watkins-Chow DE, Fu R, Borate B, Yanjanin N, Dail MK, Davidson CD, Walkley SU, Ory DS, Wassif CA, Pavan WJ, Porter FD. Microarray expression analysis and identification of serum biomarkers for Niemann-Pick disease, type C1. Human Molecular Genetics. 21: 3632-46. PMID 22619379 DOI: 10.1093/Hmg/Dds193 |
0.761 |
|
| 2012 |
Stein VM, Crooks A, Ding W, Prociuk M, O'Donnell P, Bryan C, Sikora T, Dingemanse J, Vanier MT, Walkley SU, Vite CH. Miglustat improves purkinje cell survival and alters microglial phenotype in feline Niemann-Pick disease type C. Journal of Neuropathology and Experimental Neurology. 71: 434-48. PMID 22487861 DOI: 10.1097/Nen.0B013E31825414A6 |
0.444 |
|
| 2012 |
Auclair D, Finnie J, Walkley SU, White J, Nielsen T, Fuller M, Cheng A, O'Neill CA, Hopwood JJ. Intrathecal recombinant human 4-sulfatase reduces accumulation of glycosaminoglycans in dura of mucopolysaccharidosis VI cats. Pediatric Research. 71: 39-45. PMID 22289849 DOI: 10.1038/Pr.2011.13 |
0.327 |
|
| 2012 |
Haskins M, Sleeper M, Aguirre G, Walkley SU, Knox V, Vite C, Steet R, Gurda B, Wilson J, Auricchio A, Smith L, Simonaro L, Ponder K. Large Animal Models of Lysosomal Storage Diseases: Lessons on the Limits of Gene/Enzyme Therapy Molecular Genetics and Metabolism. 105: S33-S34. DOI: 10.1016/J.Ymgme.2011.11.071 |
0.345 |
|
| 2012 |
Micsenyi MC, Walkley SU. The Lysosomal System: Physiology and Pathology Lysosomal Storage Disorders: a Practical Guide. 1-12. DOI: 10.1002/9781118514672.ch1 |
0.741 |
|
| 2011 |
Strømme P, Dobrenis K, Sillitoe RV, Gulinello M, Ali NF, Davidson C, Micsenyi MC, Stephney G, Ellevog L, Klungland A, Walkley SU. X-linked Angelman-like syndrome caused by Slc9a6 knockout in mice exhibits evidence of endosomal-lysosomal dysfunction. Brain : a Journal of Neurology. 134: 3369-83. PMID 21964919 DOI: 10.1093/Brain/Awr250 |
0.803 |
|
| 2011 |
Zhou S, Davidson C, McGlynn R, Stephney G, Dobrenis K, Vanier MT, Walkley SU. Endosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C. The American Journal of Pathology. 179: 890-902. PMID 21708114 DOI: 10.1016/J.Ajpath.2011.04.017 |
0.781 |
|
| 2011 |
Ulatowski L, Parker R, Davidson C, Yanjanin N, Kelley TJ, Corey D, Atkinson J, Porter F, Arai H, Walkley SU, Manor D. Altered vitamin E status in Niemann-Pick type C disease. Journal of Lipid Research. 52: 1400-10. PMID 21550990 DOI: 10.1194/Jlr.M015560 |
0.725 |
|
| 2011 |
Vite C, Mauldin E, Ward S, Stein V, Prociuk M, Haskins ME, Strattan R, Kao M, Ory D, Walkley SU, Vanier MT. Intrathecal cyclodextrin therapy of feline Niemann-Pick Type C disease Molecular Genetics and Metabolism. 102: S44. DOI: 10.1016/J.Ymgme.2010.11.148 |
0.413 |
|
| 2010 |
Walkley SU, Sikora J, Micsenyi M, Davidson C, Dobrenis K. Lysosomal compromise and brain dysfunction: examining the role of neuroaxonal dystrophy. Biochemical Society Transactions. 38: 1436-41. PMID 21118103 DOI: 10.1042/Bst0381436 |
0.804 |
|
| 2010 |
Curcio-Morelli C, Charles FA, Micsenyi MC, Cao Y, Venugopal B, Browning MF, Dobrenis K, Cotman SL, Walkley SU, Slaugenhaupt SA. Macroautophagy is defective in mucolipin-1-deficient mouse neurons. Neurobiology of Disease. 40: 370-7. PMID 20600908 DOI: 10.1016/J.Nbd.2010.06.010 |
0.797 |
|
| 2010 |
Davidson CD, Walkley SU. Niemann-Pick Type C Disease—Pathophysiology and Future Perspectives for Treatment Us Neurology. 6: 88. DOI: 10.17925/Usn.2010.06.01.88 |
0.702 |
|
| 2010 |
Walkley S, Ory D, Ioannou Y, Platt F, Browne R, Gallo-Hadley L, Hadley B, Hempel C, Hempel H, Jacoby J, Marella P. 142. SOAR collaborative: Progress toward therapy for a lysosomal disease through an academic research–family partnership Molecular Genetics and Metabolism. 99: S37. DOI: 10.1016/J.Ymgme.2009.10.159 |
0.379 |
|
| 2010 |
Walkley S, Davidson C, Ali N, Vanier M. 141. Cyclodextrin treatment not only delays but also reduces established intraneuronal storage in Niemann–Pick type C disease Molecular Genetics and Metabolism. 99: S37. DOI: 10.1016/J.Ymgme.2009.10.158 |
0.75 |
|
| 2010 |
Curcio-Morelli C, Charles FA, Micsenyi MC, Cao Y, Venugopal B, Browning MF, Dobrenis K, Cotman SL, Walkley SU, Slaugenhaupt SA. 34. Macroautophagy is defective in mucolipin 1-deficient mouse neurons Molecular Genetics and Metabolism. 99: S15. DOI: 10.1016/J.Ymgme.2009.10.051 |
0.772 |
|
| 2009 |
Davidson CD, Ali NF, Micsenyi MC, Stephney G, Renault S, Dobrenis K, Ory DS, Vanier MT, Walkley SU. Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression. Plos One. 4: e6951. PMID 19750228 DOI: 10.1371/Journal.Pone.0006951 |
0.808 |
|
| 2009 |
Micsenyi MC, Dobrenis K, Stephney G, Pickel J, Vanier MT, Slaugenhaupt SA, Walkley SU. Neuropathology of the Mcoln1(-/-) knockout mouse model of mucolipidosis type IV. Journal of Neuropathology and Experimental Neurology. 68: 125-35. PMID 19151629 DOI: 10.1097/Nen.0B013E3181942Cf0 |
0.799 |
|
| 2009 |
Walkley SU. Pathogenic cascades in lysosomal disease-Why so complex? Journal of Inherited Metabolic Disease. 32: 181-9. PMID 19130290 DOI: 10.1007/S10545-008-1040-5 |
0.499 |
|
| 2009 |
Walkley SU, Vanier MT. Secondary lipid accumulation in lysosomal disease. Biochimica Et Biophysica Acta. 1793: 726-36. PMID 19111580 DOI: 10.1016/J.Bbamcr.2008.11.014 |
0.459 |
|
| 2009 |
Whitley CB, Barranger JA, Eng CM, Davidson BL, Grabowski GA, Kohler B, Muenzer J, Murray GJ, Pastores GM, Patel SK, Shapiro EG, Steiner RD, Walkley SU, Wedehase BA, Wilcox WR. Lysosomal Disease Network's "WORLD Symposium 2009". Introduction. Molecular Genetics and Metabolism. 96: S3-5. PMID 19083251 DOI: 10.1016/J.Ymgme.2008.11.162 |
0.309 |
|
| 2009 |
Kaptzan T, West SA, Holicky EL, Wheatley CL, Marks DL, Wang T, Peake KB, Vance J, Walkley SU, Pagano RE. Development of a Rab9 transgenic mouse and its ability to increase the lifespan of a murine model of Niemann-Pick type C disease. The American Journal of Pathology. 174: 14-20. PMID 19056848 DOI: 10.2353/Ajpath.2009.080660 |
0.405 |
|
| 2009 |
Walkley S, Walkley SU, Dobrenis K, Micsenyi M, Cristin D. 145. The greater lysosomal system and lysosomal disease Molecular Genetics and Metabolism. 96: S44. DOI: 10.1016/J.Ymgme.2008.11.146 |
0.794 |
|
| 2009 |
Slaugenhaupt S, Curcio-Morelli C, Charles F, Venugopal B, Cao Y, Browning M, Walkley S, Pickel J, Cotman S, Purpura DP. 134. Characterization of neuronal storage in the mucolipidosis type IV murine model Molecular Genetics and Metabolism. 96: S41-S42. DOI: 10.1016/J.Ymgme.2008.11.135 |
0.388 |
|
| 2009 |
Micsenyi M, Dobrenis K, Stephney G, Walkley S. 91. P62/Sequestosome 1 accumulates in the CNS of lysosomal diseases Molecular Genetics and Metabolism. 96: S32. DOI: 10.1016/J.Ymgme.2008.11.092 |
0.781 |
|
| 2009 |
Davidson C, Walkley SU, Ali N, Stephney G. 37. Chronic cyclodextrin administration ameliorates clinical symptoms and storage accumulation in Niemann-Pick Type C1 mice Molecular Genetics and Metabolism. 96: S20. DOI: 10.1016/J.Ymgme.2008.11.038 |
0.712 |
|
| 2009 |
Blanz J, Walkley SU, Saftig P. 15. Role of LAMP2 in neuronal autophagy Molecular Genetics and Metabolism. 96: S15. DOI: 10.1016/J.Ymgme.2008.11.016 |
0.359 |
|
| 2008 |
Jabs S, Quitsch A, Käkelä R, Koch B, Tyynelä J, Brade H, Glatzel M, Walkley S, Saftig P, Vanier MT, Braulke T. Accumulation of bis(monoacylglycero)phosphate and gangliosides in mouse models of neuronal ceroid lipofuscinosis. Journal of Neurochemistry. 106: 1415-25. PMID 18498441 DOI: 10.1111/J.1471-4159.2008.05497.X |
0.486 |
|
| 2008 |
Sleat DE, El-Banna M, Sohar I, Kim KH, Dobrenis K, Walkley SU, Lobel P. Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late-infantile neuronal ceroid lipofuscinosis. Molecular Genetics and Metabolism. 94: 222-33. PMID 18343701 DOI: 10.1016/J.Ymgme.2008.01.014 |
0.47 |
|
| 2008 |
Whitley CB, Barranger JA, Eng CM, Davidson B, Grabowski GA, Muenzer J, Pastores GM, Patel SK, Shapiro EG, Steiner RD, Walkley SU, Wedehase BA, Wilcox WR. Lysosomal Disease Network's "WORLD Symposium 2008" Molecular Genetics and Metabolism. 93: S3-S5. DOI: 10.1016/J.Ymgme.2007.11.016 |
0.308 |
|
| 2007 |
Venugopal B, Browning MF, Curcio-Morelli C, Varro A, Michaud N, Nanthakumar N, Walkley SU, Pickel J, Slaugenhaupt SA. Neurologic, gastric, and opthalmologic pathologies in a murine model of mucolipidosis type IV. American Journal of Human Genetics. 81: 1070-83. PMID 17924347 DOI: 10.1086/521954 |
0.45 |
|
| 2007 |
Crawley AC, Walkley SU. Developmental analysis of CNS pathology in the lysosomal storage disease alpha-mannosidosis. Journal of Neuropathology and Experimental Neurology. 66: 687-97. PMID 17882013 DOI: 10.1097/Nen.0B013E31812503B6 |
0.47 |
|
| 2007 |
Jolly RD, Johnstone AC, Norman EJ, Hopwood JJ, Walkley SU. Pathology of mucopolysaccharidosis IIIA in Huntaway dogs. Veterinary Pathology. 44: 569-78. PMID 17846229 DOI: 10.1354/Vp.44-5-569 |
0.418 |
|
| 2007 |
Ellinwood NM, Colle MA, Weil MA, Casal ML, Vite CH, Wiemelt S, Hasson CW, O'Malley TM, He X, Prociuk U, Verot L, Melniczek JR, Lannon A, Aguirre GD, Knox VW, ... ... Walkley SU, et al. Bone marrow transplantation for feline mucopolysaccharidosis I. Molecular Genetics and Metabolism. 91: 239-50. PMID 17482862 DOI: 10.1016/J.Ymgme.2007.03.001 |
0.301 |
|
| 2007 |
Walkley SU. Pathogenic mechanisms in lysosomal disease: a reappraisal of the role of the lysosome. Acta Paediatrica (Oslo, Norway : 1992). Supplement. 96: 26-32. PMID 17391436 DOI: 10.1111/J.1651-2227.2007.00202.X |
0.38 |
|
| 2007 |
Ory D, Langmade SJ, Gale SE, Frolov A, Mohri I, Suzuki K, Mellon S, Walkley SU, Covey DF, S. J. 20 Pregnane X receptor (PXR) activation: A novel mechanism for neuroprotection in a mouse model of Niemann–Pick C disease Molecular Genetics and Metabolism. 92: 16. DOI: 10.1016/J.Ymgme.2007.08.025 |
0.357 |
|
| 2007 |
Walkley S, Gondré-Lewis M, Dobrenis K. 2 Lysosomal storage diseases: States of overabundance or deficiency? Molecular Genetics and Metabolism. 92: 11. DOI: 10.1016/J.Ymgme.2007.08.007 |
0.383 |
|
| 2007 |
Walkley SU. Lysosomal disorders of the nervous system Neurobiology of Disease. 1-18. DOI: 10.1016/B978-012088592-3/50003-7 |
0.468 |
|
| 2006 |
Langmade SJ, Gale SE, Frolov A, Mohri I, Suzuki K, Mellon SH, Walkley SU, Covey DF, Schaffer JE, Ory DS. Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C disease. Proceedings of the National Academy of Sciences of the United States of America. 103: 13807-12. PMID 16940355 DOI: 10.1073/Pnas.0606218103 |
0.431 |
|
| 2005 |
Walkley SU, Thrall MA, Haskins ME, Mitchell TW, Wenger DA, Brown DE, Dial S, Seim H. Abnormal neuronal metabolism and storage in mucopolysaccharidosis type VI (Maroteaux-Lamy) disease. Neuropathology and Applied Neurobiology. 31: 536-44. PMID 16150124 DOI: 10.1111/J.1365-2990.2005.00675.X |
0.485 |
|
| 2005 |
Narita K, Choudhury A, Dobrenis K, Sharma DK, Holicky EL, Marks DL, Walkley SU, Pagano RE. Protein transduction of Rab9 in Niemann-Pick C cells reduces cholesterol storage. Faseb Journal : Official Publication of the Federation of American Societies For Experimental Biology. 19: 1558-60. PMID 15972801 DOI: 10.1096/Fj.04-2714Fje |
0.431 |
|
| 2004 |
McGlynn R, Dobrenis K, Walkley SU. Differential subcellular localization of cholesterol, gangliosides, and glycosaminoglycans in murine models of mucopolysaccharide storage disorders. The Journal of Comparative Neurology. 480: 415-26. PMID 15558784 DOI: 10.1002/Cne.20355 |
0.496 |
|
| 2004 |
Walkley SU, Suzuki K. Consequences of NPC1 and NPC2 loss of function in mammalian neurons. Biochimica Et Biophysica Acta. 1685: 48-62. PMID 15465426 DOI: 10.1016/J.Bbalip.2004.08.011 |
0.528 |
|
| 2004 |
Walkley SU. Secondary accumulation of gangliosides in lysosomal storage disorders. Seminars in Cell & Developmental Biology. 15: 433-44. PMID 15207833 DOI: 10.1016/J.Semcdb.2004.03.002 |
0.475 |
|
| 2004 |
Sleat DE, Wiseman JA, El-Banna M, Price SM, Verot L, Shen MM, Tint GS, Vanier MT, Walkley SU, Lobel P. Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport. Proceedings of the National Academy of Sciences of the United States of America. 101: 5886-91. PMID 15071184 DOI: 10.1073/Pnas.0308456101 |
0.444 |
|
| 2004 |
Kishida T, Kostetskii I, Zhang Z, Martinez F, Liu P, Walkley SU, Dwyer NK, Blanchette-Mackie EJ, Radice GL, Strauss JF. Targeted mutation of the MLN64 START domain causes only modest alterations in cellular sterol metabolism. The Journal of Biological Chemistry. 279: 19276-85. PMID 14963026 DOI: 10.1074/Jbc.M400717200 |
0.362 |
|
| 2004 |
Stephney G, Walkley SU. Ultrastructural similarity of storage bodies in neurons lacking NPC1, NPC2, or both proteins suggests NPC1-NPC2 molecular co-operativity in lipid trafficking Microscopy and Microanalysis. 10: 1468-1469. DOI: 10.1017/S1431927604882643 |
0.367 |
|
| 2003 |
Gondré-Lewis MC, McGlynn R, Walkley SU. Cholesterol accumulation in NPC1-deficient neurons is ganglioside dependent. Current Biology : Cb. 13: 1324-9. PMID 12906793 DOI: 10.1016/S0960-9822(03)00531-1 |
0.474 |
|
| 2003 |
Walkley SU. Neurobiology and cellular pathogenesis of glycolipid storage diseases. Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 358: 893-904. PMID 12803923 DOI: 10.1098/Rstb.2003.1276 |
0.481 |
|
| 2002 |
Loftus SK, Erickson RP, Walkley SU, Bryant MA, Incao A, Heidenreich RA, Pavan WJ. Rescue of neurodegeneration in Niemann-Pick C mice by a prion-promoter-driven Npc1 cDNA transgene. Human Molecular Genetics. 11: 3107-14. PMID 12417532 DOI: 10.1093/Hmg/11.24.3107 |
0.471 |
|
| 2002 |
Jolly RD, Brown S, Das AM, Walkley SU. Mitochondrial dysfunction in the neuronal ceroid-lipofuscinoses (Batten disease). Neurochemistry International. 40: 565-71. PMID 11850114 DOI: 10.1016/S0197-0186(01)00128-0 |
0.355 |
|
| 2001 |
Walkley SU. New proteins from old diseases provide novel insights in cell biology. Current Opinion in Neurology. 14: 805-10. PMID 11723392 DOI: 10.1097/00019052-200112000-00020 |
0.414 |
|
| 2001 |
Zervas M, Somers KL, Thrall MA, Walkley SU. Critical role for glycosphingolipids in Niemann-Pick disease type C. Current Biology : Cb. 11: 1283-7. PMID 11525744 DOI: 10.1016/S0960-9822(01)00396-7 |
0.713 |
|
| 2001 |
Zervas M, Dobrenis K, Walkley SU. Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations. Journal of Neuropathology and Experimental Neurology. 60: 49-64. PMID 11202175 DOI: 10.1093/Jnen/60.1.49 |
0.695 |
|
| 2000 |
Walkley SU, Zervas M, Wiseman S. Gangliosides as modulators of dendritogenesis in normal and storage disease-affected pyramidal neurons. Cerebral Cortex (New York, N.Y. : 1991). 10: 1028-37. PMID 11007553 DOI: 10.1093/Cercor/10.10.1028 |
0.654 |
|
| 1999 |
Bhaumik M, Muller VJ, Rozaklis T, Johnson L, Dobrenis K, Bhattacharyya R, Wurzelmann S, Finamore P, Hopwood JJ, Walkley SU, Stanley P. A mouse model for mucopolysaccharidosis type III A (Sanfilippo syndrome). Glycobiology. 9: 1389-96. PMID 10561464 DOI: 10.1093/Glycob/9.12.1389 |
0.424 |
|
| 1999 |
Zervas M, Walkley SU. Ferret pyramidal cell dendritogenesis: changes in morphology and ganglioside expression during cortical development. The Journal of Comparative Neurology. 413: 429-48. PMID 10502250 DOI: 10.1002/(Sici)1096-9861(19991025)413:3<429::Aid-Cne6>3.0.Co;2-7 |
0.593 |
|
| 1999 |
Jolly RD, Walkley SU. Ovine ceroid lipofuscinosis (OCL6): postulated mechanism of neurodegeneration. Molecular Genetics and Metabolism. 66: 376-80. PMID 10191132 DOI: 10.1006/Mgme.1999.2821 |
0.425 |
|
| 1999 |
Walkley SU, Zervas M, Siegel DA, Dobrenis K. Gangliosides as modulators of dendritogenesis in storage disease- affected and normal pyramidal neurons Developmental Neuropsychology. 16: 361-364. DOI: 10.1207/S15326942Dn1603_16 |
0.671 |
|
| 1999 |
Jolly RD, Kohlschütter A, Palmer DN, Walkley SU. Chapter 11 The Neuronal Ceroid-lipofuscinoses (Batten Disease) Advances in Cell Aging and Gerontology. 3: 391-420. DOI: 10.1016/S1566-3124(08)60031-0 |
0.351 |
|
| 1998 |
Walkley SU, Siegel DA, Dobrenis K, Zervas M. GM2 ganglioside as a regulator of pyramidal neuron dendritogenesis. Annals of the New York Academy of Sciences. 845: 188-99. PMID 9668352 DOI: 10.1111/J.1749-6632.1998.Tb09671.X |
0.656 |
|
| 1998 |
Walkley SU. Cellular pathology of lysosomal storage disorders. Brain Pathology (Zurich, Switzerland). 8: 175-93. PMID 9458175 DOI: 10.1111/J.1750-3639.1998.Tb00144.X |
0.503 |
|
| 1997 |
Jolly RD, Walkley SU. Lysosomal storage diseases of animals: an essay in comparative pathology. Veterinary Pathology. 34: 527-48. PMID 9396134 |
0.362 |
|
| 1997 |
March PA, Thrall MA, Brown DE, Mitchell TW, Lowenthal AC, Walkley SU. GABAergic neuroaxonal dystrophy and other cytopathological alterations in feline Niemann-Pick disease type C. Acta Neuropathologica. 94: 164-72. PMID 9255392 DOI: 10.1007/S004010050689 |
0.461 |
|
| 1996 |
Goodman LA, Walkley SU. Elevated GM2 ganglioside is associated with dendritic proliferation in normal developing neocortex. Brain Research. Developmental Brain Research. 93: 162-71. PMID 8804703 DOI: 10.1016/0165-3806(96)00029-6 |
0.402 |
|
| 1996 |
Brown DE, Thrall MA, Walkley SU, Wurzelmann S, Wenger DA, Allison RW, Just CA. Metabolic abnormalities in feline Niemann-Pick type C heterozygotes. Journal of Inherited Metabolic Disease. 19: 319-30. PMID 8803775 DOI: 10.1007/Bf01799262 |
0.441 |
|
| 1995 |
Walkley SU, Siegel DA, Dobrenis K. GM2 ganglioside and pyramidal neuron dendritogenesis. Neurochemical Research. 20: 1287-99. PMID 8786814 DOI: 10.1007/Bf00992503 |
0.478 |
|
| 1995 |
Walkley SU. Pyramidal neurons with ectopic dendrites in storage diseases exhibit increased GM2 ganglioside immunoreactivity. Neuroscience. 68: 1027-35. PMID 8544979 DOI: 10.1016/0306-4522(95)00208-Z |
0.489 |
|
| 1995 |
March PA, Wurzelmann S, Walkley SU. Morphological alterations in neocortical and cerebellar GABAergic neurons in a canine model of juvenile Batten disease. American Journal of Medical Genetics. 57: 204-12. PMID 7668331 DOI: 10.1002/Ajmg.1320570219 |
0.41 |
|
| 1995 |
Walkley SU, March PA, Schroeder CE, Wurzelmann S, Jolly RD. Pathogenesis of brain dysfunction in Batten disease. American Journal of Medical Genetics. 57: 196-203. PMID 7668330 DOI: 10.1002/Ajmg.1320570218 |
0.515 |
|
| 1995 |
Walkley SU, Wurzelmann S. Alterations in synaptic connectivity in cerebral cortex in neuronal storage disorders Mental Retardation and Developmental Disabilities Research Reviews. 1: 183-192. DOI: 10.1002/Mrdd.1410010306 |
0.469 |
|
| 1994 |
Brown DE, Thrall MA, Walkley SU, Wenger DA, Mitchell TW, Smith MO, Royals KL, March PA, Allison RW. Feline Niemann-Pick disease type C. The American Journal of Pathology. 144: 1412-5. PMID 8203477 |
0.324 |
|
| 1994 |
Walkley SU, Thrall MA, Dobrenis K, Huang M, March PA, Siegel DA, Wurzelmann S. Bone marrow transplantation corrects the enzyme defect in neurons of the central nervous system in a lysosomal storage disease. Proceedings of the National Academy of Sciences of the United States of America. 91: 2970-4. PMID 8159689 DOI: 10.1073/Pnas.91.8.2970 |
0.442 |
|
| 1994 |
Siegel DA, Walkley SU. Growth of ectopic dendrites on cortical pyramidal neurons in neuronal storage diseases correlates with abnormal accumulation of GM2 ganglioside. Journal of Neurochemistry. 62: 1852-62. PMID 8158134 DOI: 10.1046/J.1471-4159.1994.62051852.X |
0.43 |
|
| 1993 |
Walkley SU, March PA. Biology of neuronal dysfunction in storage disorders. Journal of Inherited Metabolic Disease. 16: 284-7. PMID 8411982 DOI: 10.1007/Bf00710266 |
0.358 |
|
| 1993 |
March PA, Walkley SU, Wurzelmann S. MITOCHONDRIAL ABNORMALITIES WITHIN A DISTINCT POPULATION OF NON-PYRAMIDAL NEURONS IN BATTENʼS DISEASE Journal of Neuropathology and Experimental Neurology. 52: 281. DOI: 10.1097/00005072-199305000-00086 |
0.402 |
|
| 1991 |
Walkley SU, Baker HJ, Rattazzi MC, Haskins ME, Wu JY. Neuroaxonal dystrophy in neuronal storage disorders: evidence for major GABAergic neuron involvement. Journal of the Neurological Sciences. 104: 1-8. PMID 1919594 DOI: 10.1016/0022-510X(91)90208-O |
0.435 |
|
| 1991 |
Goodman LA, Livingston PO, Walkley SU. Ectopic dendrites occur only on cortical pyramidal cells containing elevated GM2 ganglioside in alpha-mannosidosis. Proceedings of the National Academy of Sciences of the United States of America. 88: 11330-4. PMID 1763046 DOI: 10.1073/Pnas.88.24.11330 |
0.39 |
|
| 1990 |
Walkley SU, Wurzelmann S, Rattazzi MC, Baker HJ. Distribution of ectopic neurite growth and other geometrical distortions of CNS neurons in feline GM2 gangliosidosis. Brain Research. 510: 63-73. PMID 2322847 DOI: 10.1016/0006-8993(90)90728-T |
0.452 |
|
| 1990 |
Walkley SU, Baker HJ, Rattazzi MC. Initiation and growth of ectopic neurites and meganeurites during postnatal cortical development in ganglioside storage disease. Brain Research. Developmental Brain Research. 51: 167-78. PMID 2108821 DOI: 10.1016/0165-3806(90)90273-2 |
0.414 |
|
| 1988 |
Cummings JF, Wood PA, de Lahunta A, Walkley SU, Le Boeuf L. The clinical and pathologic heterogeneity of feline alpha-mannosidosis. Journal of Veterinary Internal Medicine / American College of Veterinary Internal Medicine. 2: 163-70. PMID 3230555 DOI: 10.1111/J.1939-1676.1988.Tb00311.X |
0.306 |
|
| 1988 |
Ahern-Rindell AJ, Prieur DJ, Murnane RD, Raghavan SS, Daniel PF, McCluer RH, Walkley SU, Parish SM. Inherited lysosomal storage disease associated with deficiencies of beta-galactosidase and alpha-neuraminidase in sheep. American Journal of Medical Genetics. 31: 39-56. PMID 3146925 DOI: 10.1002/Ajmg.1320310108 |
0.344 |
|
| 1988 |
Walkley SU, Siegel DA, Wurzelmann S. Ectopic dendritogenesis and associated synapse formation in swainsonine-induced neuronal storage disease. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 8: 445-57. PMID 3123615 DOI: 10.1523/Jneurosci.08-02-00445.1988 |
0.476 |
|
| 1988 |
Walkley SU. Pathobiology of neuronal storage disease. International Review of Neurobiology. 29: 191-244. PMID 3042663 DOI: 10.1016/S0074-7742(08)60087-2 |
0.475 |
|
| 1988 |
Walkley SU, Haskins ME, Shull RM. Alterations in neuron morphology in mucopolysaccharidosis type I. A Golgi study. Acta Neuropathologica. 75: 611-20. PMID 2454013 DOI: 10.1007/Bf00686207 |
0.453 |
|
| 1987 |
Baker HJ, Wood PA, Wenger DA, Walkley SU, Inui K, Kudoh T, Rattazzi MC, Riddle BL. Sphingomyelin lipidosis in a cat. Veterinary Pathology. 24: 386-91. PMID 3672804 DOI: 10.1177/030098588702400504 |
0.425 |
|
| 1987 |
Walkley SU. Further studies on ectopic dendrite growth and other geometrical distortions of neurons in feline GM1 gangliosidosis. Neuroscience. 21: 313-31. PMID 3112606 DOI: 10.1016/0306-4522(87)90124-2 |
0.437 |
|
| 1987 |
Walkley SU, Wurzelmann S, Siegel DA. Ectopic axon hillock-associated neurite growth is maintained in metabolically reversed swainsonine-induced neuronal storage disease. Brain Research. 410: 89-96. PMID 3107757 DOI: 10.1016/S0006-8993(87)80025-2 |
0.445 |
|
| 1986 |
Walkley SU, Pierok AL. Ferric ion-ferrocyanide staining in ganglioside storage disease establishes that meganeurites are of axon hillock origin and distinct from axonal spheroids. Brain Research. 382: 379-86. PMID 2428446 DOI: 10.1016/0006-8993(86)91348-X |
0.306 |
|
| 1985 |
Walkley SU, Siegel DA. Ectopic dendritogenesis occurs on cortical pyramidal neurons in swainsonine-induced feline alpha-mannosidosis. Brain Research. 352: 143-8. PMID 3924348 DOI: 10.1016/0165-3806(85)90098-7 |
0.4 |
|
| 1985 |
Karabelas AB, Walkley SU. Altered patterns of evoked synaptic activity in cortical pyramidal neurons in feline ganglioside storage disease. Brain Research. 339: 329-36. PMID 2992703 DOI: 10.1016/0006-8993(85)90099-X |
0.376 |
|
| 1985 |
Cummings JF, Wood PA, Walkley SU, de Lahunta A, DeForest ME. GM2 gangliosidosis in a Japanese spaniel. Acta Neuropathologica. 67: 247-53. PMID 2931941 DOI: 10.1007/Bf00687809 |
0.37 |
|
| 1984 |
Walkley SU, James LF. Locoweed-induced neuronal storage disease characterized by meganeurite formation. Brain Research. 324: 145-50. PMID 6518385 DOI: 10.1016/0006-8993(84)90633-4 |
0.414 |
|
| 1984 |
Walkley SU, Baker HJ. Sphingomyelin lipidosis in a cat: Golgi studies. Acta Neuropathologica. 65: 138-44. PMID 6441439 DOI: 10.1007/Bf00690467 |
0.418 |
|
| 1982 |
Huxtable CR, Dorling PR, Walkley SU. Onset and regression of neuroaxonal lesions in sheep with mannosidosis induced experimentally with swainsonine. Acta Neuropathologica. 58: 27-33. PMID 6814163 DOI: 10.1007/Bf00692694 |
0.374 |
|
| 1981 |
Walkley SU, Blakemore WF, Purpura DP. Alterations in neuron morphology in feline mannosidosis. A Golgi study. Acta Neuropathologica. 53: 75-9. PMID 7211201 DOI: 10.1007/Bf00697187 |
0.644 |
|
| 1981 |
Walkley SU, Wurzelmann S, Purpura DP. Ultrastructure of neurites and meganeurites of cortical pyramidal neurons in feline gangliosidosis as revealed by the combined Golgi-EM technique. Brain Research. 211: 393-8. PMID 6165433 DOI: 10.1016/0006-8993(81)90711-3 |
0.63 |
|
| 1980 |
Purpura DP, Highstein SM, Karabelas AB, Walkley SU. Intracellular recording and HRP-staining of cortical neurons in feline ganglioside storage disease. Brain Research. 181: 446-9. PMID 6766075 DOI: 10.1016/0006-8993(80)90627-7 |
0.653 |
|
| 1979 |
Baker HJ, Reynolds GD, Walkley SU, Cox NR, Baker GH. The gangliosidoses: comparative features and research applications. Veterinary Pathology. 16: 635-49. PMID 116415 DOI: 10.1177/030098587901600602 |
0.37 |
|
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