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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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Detalhes bibliográficos
Main Authors: Zampieri, Stefania, Mellon, Synthia H, Butters, Terry D, Nevyjel, Marco, Covey, Douglas F, Bembi, Bruno, Dardis, Andrea
Formato: Artigo
Idioma:Inglês
Publicado em: John Wiley & Sons, Ltd 2009
Assuntos:
Acesso em linha: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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