- 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....
Expand abstract - Publication status:
- Published
- Publisher copy:
- 10.1111/j.1582-4934.2008.00493.x
-
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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
- Copyright date:
- 2009
Journal article
Oxidative stress in NPC1 deficient cells: protective effect of allopregnanolone.
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