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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Bibliografske podrobnosti
Main Authors: Zampieri, Stefania, Mellon, Synthia H, Butters, Terry D, Nevyjel, Marco, Covey, Douglas F, Bembi, Bruno, Dardis, Andrea
Format: Artigo
Jezik:Inglês
Izdano: John Wiley & Sons, Ltd 2009
Teme:
Molecular Medicine
Online dostop: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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