• 5703 Citations
  • 44 h-Index
1976 …2022
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Fingerprint Dive into the research topics where Steven U. Walkley is active. These topic labels come from the works of this person. Together they form a unique fingerprint.

  • 2 Similar Profiles
Type C Niemann-Pick Disease Medicine & Life Sciences
Neurons Medicine & Life Sciences
Felidae Medicine & Life Sciences
G(M2) Ganglioside Medicine & Life Sciences
Gangliosides Medicine & Life Sciences
Pyramidal Cells Medicine & Life Sciences
Gangliosidoses Medicine & Life Sciences
Lysosomal Storage Diseases Medicine & Life Sciences

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Grants 1977 2022

Developmental Disabilities
Cell Engineering
Intellectual Disability
Research
leadership

Histone Deacetylase Inhibitors for Treatment of Niemann-Pick C1 Disease

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

National Institutes of Health

9/15/157/31/20

Project: Research project

Niemann-Pick Diseases
Histone Deacetylase Inhibitors
Mutation
Mutant Proteins
Therapeutics
Lysosomes
Autophagy
Neuroaxonal Dystrophies
Proteins
Dendrites
Seizures
Epilepsy
Research
Mental Retardation, X-Linked, Syndromic, Christianson Type
Endosomes
Research
Research Personnel
Students
Investigational Therapies
Health

Publications 1976 2019

  • 5703 Citations
  • 44 h-Index
  • 117 Article
  • 2 Chapter
  • 1 Editorial
  • 1 Review article

2-Hydroxypropyl-β-cyclodextrin is the active component in a triple combination formulation for treatment of Niemann-Pick C1 disease

Davidson, J., Molitor, E., Moores, S., Gale, S. E., Subramanian, K., Jiang, X., Sidhu, R., Kell, P., Zhang, J., Fujiwara, H., Davidson, C., Helquist, P., Melancon, B. J., Grigalunas, M., Liu, G., Salahi, F., Wiest, O., Xu, X., Porter, F. D., Pipalia, N. H. & 6 others, Cruz, D. L., Holson, E. B., Schaffer, J. E., Walkley, S. U., Maxfield, F. R. & Ory, D. S., Jan 1 2019, In : Biochimica et Biophysica Acta - Molecular and Cell Biology of Lipids.

Research output: Contribution to journalArticle

Niemann-Pick Diseases
Histone Deacetylase Inhibitors
Cyclodextrins
Pharmacokinetics
Type C Niemann-Pick Disease
4 Citations (Scopus)

Current concepts in the neuropathogenesis of mucolipidosis type IV

Boudewyn, L. C. & Walkley, S. U., Jan 1 2018, (Accepted/In press) In : Journal of Neurochemistry.

Research output: Contribution to journalArticle

Mucolipidoses
Brain
Transient Receptor Potential Channels
Haploinsufficiency
Retinal Degeneration
1 Citation (Scopus)
Intellectual Disability
Nervous System
Spinal Cord
G(M2) Ganglioside
Phenotype
13 Citations (Scopus)

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: Contribution to journalArticle

Farber Lipogranulomatosis
Nervous System Malformations
Ceramides
Central Nervous System
Microglia
12 Citations (Scopus)

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: Contribution to journalArticle

Autophagy
Cyclodextrins
Cathepsin D
Brain
Lysosomes