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Grants 1977 2021

Support for the Rose F. Kennedy IDDRC

Morrow, B. E., Schwartz, G. J., Greally, J. M., Dobrenis, K., Walkley, S. U. & Molholm, S.

National Institutes of Health

9/22/165/31/21

Project: Research projectSpecialized Center--Cooperative Agreements

Intellectual Disability
Research
DiGeorge Syndrome
Developmental Disabilities
Cell Engineering

Histone Deacetylase Inhibitors for Treatment of Niemann-Pick C1 Disease

Walkley, S. U., Ory, D., Maxfield, F. R. & Maxfield, F. R.

National Institutes of Health

9/15/157/31/20

Project: Research projectResearch Project

Niemann-Pick Diseases
Histone Deacetylase Inhibitors
Therapeutics
Mutation
Mutant Proteins
Mental Retardation, X-Linked, Syndromic, Christianson Type
Seizures
Research
Epilepsy
Endosomes
Research
Research Personnel
Students
Health
Brain
Research
Chronic Disease
Research Personnel
Students
Brain

Publications 1976 2017

  • 4879 Citations
  • 39 h-Index
  • 117 Article
  • 2 Chapter
  • 1 Review article
1 Citations

Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities

Sikora, J., Dworski, S., Jones, E. E., Kamani, M. A., Micsenyi, M. C., Sawada, T., Le Faouder, P., Bertrand-Michel, J., Dupuy, A., Dunn, C. K., Xuan, I. C. Y., Casas, J., Fabrias, G., Hampson, D. R., Levade, T., Drake, R. R., Medin, J. A. & Walkley, S. U. Apr 1 2017 In : American Journal of Pathology. 187, 4, p. 864-883 20 p.

Research output: Research - peer-reviewArticle

Farber Lipogranulomatosis
Nervous System Malformations
Central Nervous System
Ceramides
Microglia
3 Citations

Cyclodextrin has conflicting actions on autophagy flux in vivo in brains of normal and Alzheimer model mice

Yang, D. S., Stavrides, P., Kumar, A., Jiang, Y., Mohan, P. S., Ohno, M., Dobrenis, K., Davidson, C. D., Saito, M., Pawlik, M., Huo, C., Walkley, S. U. & Nixon, R. A. Mar 1 2017 In : Human Molecular Genetics. 26, 5, p. 843-859 17 p., ddx001

Research output: Research - peer-reviewArticle

Autophagy
Cyclodextrins
Brain
Autophagosomes
Cathepsin D

Intrathecal 2-hydroxypropyl-β-cyclodextrin decreases neurological disease progression in Niemann-Pick disease, type C1: A non-randomised, open-label, phase 1-2 trial

Ory, D. S. , Ottinger, E. A. , Farhat, N. Y. , King, K. A. , Jiang, X. , Weissfeld, L. , Berry-Kravis, E. , Davidson, C. D. , Bianconi, S. , Keener, L. A. , Rao, R. , Soldatos, A. , Sidhu, R. , Walters, K. A. , Xu, X. , Thurm, A. , Solomon, B. , Pavan, W. J. , Machielse, B. N. , Kao, M. & 7 others Silber, S. A., McKew, J. C., Brewer, C. C., Vite, C. H., Walkley, S. U., Austin, C. P. & Porter, F. D. 2017 (Accepted/In press) In : The Lancet.

Research output: Research - peer-reviewArticle

Type A Niemann-Pick Disease
Type C Niemann-Pick Disease
Disease Progression
2-hydroxypropyl-beta-cyclodextrin
National Institutes of Health (U.S.)

N-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV

Boudewyn, L. C., Sikora, J., Kuchar, L., Ledvinova, J., Grishchuk, Y., Wang, S. L., Dobrenis, K. & Walkley, S. U. Sep 1 2017 In : Neurobiology of Disease. 105, p. 257-270 14 p.

Research output: Research - peer-reviewArticle

Mucolipidoses
Purkinje Cells
miglustat
Glycosphingolipids
Pathology

Niemann-Pick disease, type C and Roscoe Brady

Patterson, M. C. & Walkley, S. U. Jan 1 2017 In : Molecular Genetics and Metabolism. 120, 1-2, p. 34-37 4 p.

Research output: Research - peer-reviewReview article

Type C Niemann-Pick Disease
Type B Niemann-Pick Disease
Type A Niemann-Pick Disease
Niemann-Pick Diseases
Sphingomyelin Phosphodiesterase