298 results on '"Walkley, Steven U."'
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2. Free sialic acid storage disorder: Progress and promise
3. Intellectual and developmental disabilities research centers: Fifty years of scientific accomplishments
4. Genetic Evidence for Nonredundant Functional Cooperativity between NPC1 and NPC2 in Lipid Transport
5. Neurobiology and Cellular Pathogenesis of Glycolipid Storage Diseases
6. Intrathecal 2-hydroxypropyl-β-cyclodextrin decreases neurological disease progression in Niemann-Pick disease, type C1: a non-randomised, open-label, phase 1–2 trial
7. N-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV
8. A concerted action to explore therapies for free sialic acid storage disease
9. The Lysosomal System
10. Cyclodextrin has conflicting actions on autophagy flux in vivo in brains of normal and Alzheimer model mice
11. Batten Disease: A Typical Neuronal Storage Disease or a Genetic Neurodegenerative Disorder Characterized by Excitotoxicity?
12. Endosomal/Lysosomal Processing of Gangliosides Affects Neuronal Cholesterol Sequestration in Niemann-Pick Disease Type C
13. A concerted action to explore therapies for free sialic acid storage disease (FSASD)
14. Pregnane X Receptor (PXR) Activation: A Mechanism for Neuroprotection in a Mouse Model of Niemann-Pick C Disease
15. Rethinking lysosomes and lysosomal disease
16. Ataxia is the major neuropathological finding in arylsulfatase G-deficient mice: similarities and dissimilarities to Sanfilippo disease (mucopolysaccharidosis type III)
17. Defective macroautophagic turnover of brain lipids in the TgCRND8 Alzheimer mouse model: prevention by correcting lysosomal proteolytic deficits
18. Free sialic acid storage disorder: Progress and promise
19. Neurologic, gastric, and opthalmologic pathologies in a murine model of mucolipidosis type IV
20. The Lysosomal System
21. Microarray expression analysis and identification of serum biomarkers for Niemann–Pick disease, type C1
22. Miglustat Improves Purkinje Cell Survival and Alters Microglial Phenotype in Feline Niemann-Pick Disease Type C
23. X-linked Angelman-like syndrome caused by Slc9a6 knockout in mice exhibits evidence of endosomal–lysosomal dysfunction
24. Cerebellar Alterations and Gait Defects as Therapeutic Outcome Measures for Enzyme Replacement Therapy in α-Mannosidosis
25. Lysosomal Disorders of the Nervous System
26. Contributors
27. Neuropathology of the Mcoln1−/− Knockout Mouse Model of Mucolipidosis Type IV
28. Pathogenic cascades and brain dysfunction
29. Developmental Analysis of CNS Pathology in the Lysosomal Storage Disease α-Mannosidosis
30. Pathogenic mechanisms in lysosomal disease: a reappraisal of the role of the lysosome
31. GM2 ganglioside and pyramidal neuron dendritogenesis
32. Rescue of neurodegeneration in Niemann–Pick C mice by a prion-promoter-driven Npc1 cDNA transgene
33. Neurons in Niemann-Pick Disease Type C Accumulate Gangliosides as Well as Unesterified Cholesterol and Undergo Dendritic and Axonal Alterations
34. 2-Hydroxypropyl-β-cyclodextrin is the active component in a triple combination formulation for treatment of Niemann-Pick C1 disease
35. Improved disease amelioration with combination therapy for Niemann-Pick disease type C1
36. GM2 Ganglioside as a Regulator of Pyramidal Neuron Dendritogenesisa
37. Current concepts in the neuropathogenesis of mucolipidosis type IV
38. Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities
39. Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities
40. Niemann-Pick disease, type C and Roscoe Brady
41. Fostering collaborative research for rare genetic disease: the example of niemann-pick type C disease
42. Current concepts in the neuropathogenesis of mucolipidosis type IV.
43. Collaborative Development of 2-Hydroxypropyl-β-Cyclodextrin for the Treatment of Niemann-Pick Type C1 Disease
44. Efficacy and ototoxicity of different cyclodextrins in Niemann–Pick C disease
45. Meganeurites and The Man
46. Multisensory processing in isorders: A behavioral and high-density electrophysiology investigation in Niemann-Pick type C and cystinosis
47. Different cyclodextrins for the treatment of Niemann-Pick disease type C
48. Phase 1/2 evaluation of intrathecal 2-hydroxypropyl-β-cyclodextrin for the treatment of Niemann-Pick disease type C1
49. Assessment of n-butyl-deoxynojirimycin as a therapeutic option for mucolipidosis type IV
50. Niemann-Pick C1 Is Essential for Ebolavirus Replication and Pathogenesis In Vivo
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