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...

Deskribapen osoa

Gorde:
Xehetasun bibliografikoak
Argitaratua izan da:Redox Biol
Egile Nagusiak: 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
Formatua: Artigo
Hizkuntza:Inglês
Argitaratua: Elsevier 2021
Gaiak:
Research Paper
Sarrera elektronikoa: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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