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Career

· Research Scientist, Carnegie Mellon University

Publications (42)

Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium
Nature Medicine · 2008
832
cited
Heat shock protein–based therapy as a potential candidate for treating the sphingolipidoses
Science Translational Medicine · 2016
155
cited
Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1
Neurobiology of Disease · 2009
144
cited
NSAIDs increase survival in the Sandhoff disease mouse: Synergy with N‐butyldeoxynojirimycin
Annals of Neurology · 2004
123
cited
Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann–Pick type C1 mice
Neurobiology of Disease · 2012
95
cited
Macroautophagy Is Not Directly Involved in the Metabolism of Amyloid Precursor Protein*
Journal of Biological Chemistry · 2010
94
cited
Relative acidic compartment volume as a lysosomal storage disorder-associated biomarker.
Journal of Clinical Investigation · 2014
83
cited
Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis.
Molecular Genetics and Metabolism · 2008
82
cited
Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease
Neurobiology of Disease · 2004
80
cited
Improved neuroprotection using miglustat, curcumin and ibuprofen as a triple combination therapy in Niemann–Pick disease type C1 mice
Neurobiology of Disease · 2014
78
cited
Acetyl-leucine slows disease progression in lysosomal storage disorders
bioRxiv · 2020
69
cited
Immune dysfunction in Niemann‐Pick disease type C
Journal of Neurochemistry · 2015
66
cited
AAV9 intracerebroventricular gene therapy improves lifespan, locomotor function and pathology in a mouse model of Niemann–Pick type C1 disease
Human Molecular Genetics · 2018
53
cited
Neural Stem Cell Transplantation Benefits a Monogenic Neurometabolic Disorder During the Symptomatic Phase of Disease
Stem Cells · 2009
48
cited
Beneficial Effects of Acetyl-DL-Leucine (ADLL) in a Mouse Model of Sandhoff Disease
Journal of Clinical Medicine · 2020
41
cited
Altered distribution and function of natural killer cells in murine and human Niemann-Pick disease type C1.
Blood · 2014
35
cited
Critical role of iron in the pathogenesis of the murine gangliosidoses
Neurobiology of Disease · 2009
32
cited
An Inducible Mouse Model of Late Onset Tay–Sachs Disease
Neurobiology of Disease · 2002
30
cited
Defective Cytochrome P450-Catalysed Drug Metabolism in Niemann-Pick Type C Disease
PLoS ONE · 2016
23
cited
Invariant natural killer T cells are not affected by lysosomal storage in patients with Niemann‐Pick disease type C
European Journal of Immunology · 2012
18
cited
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Dr. David A. Smith | Researcher Profile | Sotabase | Sotabase