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...

詳細記述

書誌詳細
主要な著者: Zampieri, S, Mellon, S, Butters, T, Nevyjel, M, Covey, D, Bembi, B, Dardis, A
フォーマット: Journal article
言語:English
出版事項: 2009

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