Acid ceramidase improves mitochondrial function and oxidative stress in Niemann-Pick type C disease by repressing STARD1 expression and mitochondrial cholesterol accumulation

Acid ceramidase improves mitochondrial function and oxidative stress in Niemann-Pick type C disease by repressing STARD1 expression and mitochondrial cholesterol accumulation

Niemann-Pick type C (NPC) disease, a lysosomal storage disorder caused by defective NPC1/NPC2 function, results in the accumulation of cholesterol and glycosphingolipids in lysosomes of affected organs, such as liver and brain. Moreover, increase of mitochondrial cholesterol (mchol) content and impa...

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Bibliographic Details
Main Authors: Sandra Torres, Estel Solsona-Vilarrasa, Susana Nuñez, Nuria Matías, Naroa Insausti-Urkia, Fernanda Castro, Mireia Casasempere, Gemma Fabriás, Josefina Casas, Carlos Enrich, José C. Fernández-Checa, Carmen Garcia-Ruiz
Format: Article
Language:English
Published: Elsevier 2021-09-01
Series:Redox Biology
Subjects:
Cholesterol
Acid ceramiase
Mitochondrial function
Oxidative stress
NPC disease
Online Access:http://www.sciencedirect.com/science/article/pii/S2213231721002111

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http://www.sciencedirect.com/science/article/pii/S2213231721002111

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