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Journal article

Oxidative stress in NPC1 deficient cells: protective effect of allopregnanolone.

Abstract:

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 disease are not clear. Oxidative damage is implicated in the pathophysiology of different neurological disorders and the effect of GSL accumulation on the intracellular redox state has been documented....

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Publication status:
Published

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Authors


Zampieri, S More by this author
Mellon, SH More by this author
Butters, TD More by this author
Nevyjel, M More by this author
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Journal:
Journal of cellular and molecular medicine
Volume:
13
Issue:
9B
Pages:
3786-3796
Publication date:
2009-09-05
DOI:
EISSN:
1582-4934
ISSN:
1582-1838
URN:
uuid:d6d3cbea-6dc3-4fa5-a183-93475f8d7d4c
Source identifiers:
101134
Local pid:
pubs:101134

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