S
S. Joshua Langmade
Researcher at Washington University in St. Louis
Publications - 5
Citations - 744
S. Joshua Langmade is an academic researcher from Washington University in St. Louis. The author has contributed to research in topics: NPC1 & Pregnane X receptor. The author has an hindex of 4, co-authored 5 publications receiving 686 citations.
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Journal ArticleDOI
Cholesterol Oxidation Products Are Sensitive and Specific Blood-Based Biomarkers for Niemann-Pick C1 Disease
Forbes D. Porter,David E. Scherrer,Michael H. Lanier,S. Joshua Langmade,Vasumathi Molugu,Sarah E. Gale,Dana Olzeski,Rohini Sidhu,Dennis J. Dietzen,Rao Fu,Christopher A. Wassif,Nicole M. Yanjanin,Steven P. Marso,John A. House,Charles H. Vite,Jean E. Schaffer,Daniel S. Ory +16 more
TL;DR: Blood concentrations of two related oxysterols molecules were almost 10 times higher in Niemann-Pick C1 patients than in age-matched healthy controls or those with other diseases such as atherosclerosis or diabetes, suggesting that the two oxysterol molecules are accurate diagnostic markers of early clinical disease and can be used not only to monitor disease progression but also to demonstrate drug efficacy.
Journal ArticleDOI
Niemann-Pick Type C1 I1061T Mutant Encodes a Functional Protein That Is Selected for Endoplasmic Reticulum-associated Degradation Due to Protein Misfolding *
Mark E. Gelsthorpe,Nikola A. Baumann,Elizabeth E. Millard,Sarah E. Gale,S. Joshua Langmade,Jean E. Schaffer,Daniel S. Ory +6 more
TL;DR: These findings provide the first description of an endoplasmic reticulum trafficking defect as a mechanism for human NPC disease, shedding light on the mechanism by which the NPC1I1061T mutation causes disease and suggesting novel approaches to treat NPC disease caused by the NPC 1I10 61T mutation.
Journal ArticleDOI
Pregnane X receptor (PXR) activation: A mechanism for neuroprotection in a mouse model of Niemann-Pick C disease
S. Joshua Langmade,Sarah E. Gale,Andrey Frolov,Ikuko Mohri,Kinuko Suzuki,Synthia H. Mellon,Steven U. Walkley,Douglas F. Covey,Jean E. Schaffer,Daniel S. Ory +9 more
TL;DR: It is shown that the neurosteroid allopregnanolone (ALLO) and T0901317, a synthetic oxysterol ligand, act in concert to delay onset of neurological symptoms and prolong the lifespan of npc1−/− mice, suggesting that treatment with pregnane X receptor ligands may be useful clinically in delaying the progressive neurodegeneration in human NPC disease.
Journal ArticleDOI
Niemann-Pick C1 protects against atherosclerosis in mice via regulation of macrophage intracellular cholesterol trafficking.
Jessie Zhang,Trey Coleman,S. Joshua Langmade,David E. Scherrer,Lindsay Lane,M. Hunter Lanier,Chu Feng,Mark S. Sands,Jean E. Schaffer,Clay F. Semenkovich,Daniel S. Ory +10 more
TL;DR: The results demonstrate that NPC1 serves an atheroprotective role in mice through regulation of LXR-dependent cholesterol efflux and mitigation of cholesterol-induced oxidative stress in macrophages.