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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Dettagli Bibliografici
Pubblicato in:Redox Biol
Autori principali: Torres, Sandra, Solsona-Vilarrasa, Estel, Nuñez, Susana, Matías, Nuria, Insausti-Urkia, Naroa, Castro, Fernanda, Casasempere, Mireia, Fabriás, Gemma, Casas, Josefina, Enrich, Carlos, Fernández-Checa, José C., Garcia-Ruiz, Carmen
Natura: Artigo
Lingua:Inglês
Pubblicazione: Elsevier 2021
Soggetti:
Research Paper
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC8254009/
https://ncbi.nlm.nih.gov/pubmed/34175669
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.redox.2021.102052
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