Article
Altered vitamin E status in Niemann-Pick type C disease.
Journal of lipid research - 1 Jul 2011
Ulatowski L, Parker R, Davidson C, Yanjanin N, Kelley T J, Corey D, Atkinson J, Porter F, Arai H, Walkley S U, Manor D
Abstract excerpt
Vitamin E (α-tocopherol) is the major lipid-soluble antioxidant in many species. Niemann-Pick type C (NPC) disease is a lysosomal storage disorder caused by mutations in the NPC1 or NPC2 gene, which regulates lipid transport through the endocytic pathway. NPC disease is characterized by massive intracellular accumulation of unesterified cholesterol and other lipids in lysosomal vesicles. We examined the roles...
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