Oxidative stress in NPC1 deficient cells: protective effect of allopregnanolone

Niemann-Pick C disease (NPC) is an autosomal recessive neurodegenerative disorder caused by the abnormal function of NPC1 or NPC2 proteins, leading to an accumulation of unesterified cholesterol and glycosphingolipids (GSLs) in the lysosomes. The mechanisms underlying the pathophysiology in NPC dise...

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Bibliografiske detaljer
Main Authors: Zampieri, Stefania, Mellon, Synthia H, Butters, Terry D, Nevyjel, Marco, Covey, Douglas F, Bembi, Bruno, Dardis, Andrea
Format: Artigo
Sprog:Inglês
Udgivet: John Wiley & Sons, Ltd 2009
Fag:
Molecular Medicine
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC2832077/
https://ncbi.nlm.nih.gov/pubmed/18774957
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1111/j.1582-4934.2008.00493.x
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