Leigh Syndrome Mouse Model Can Be Rescued by Interventions that Normalize Brain Hyperoxia, but Not HIF Activation

Leigh syndrome is a devastating mitochondrial disease for which there are no proven therapies. We previously showed that breathing chronic, continuous hypoxia can prevent and even reverse neurological disease in the Ndufs4 knockout (KO) mouse model of complex I (CI) deficiency and Leigh syndrome. He...

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Publicat a:Cell Metab
Autors principals: Jain, Isha H., Zazzeron, Luca, Goldberger, Olga, Marutani, Eizo, Wojtkiewicz, Gregory R., Ast, Tslil, Wang, Hong, Schleifer, Grigorij, Stepanova, Anna, Brepoels, Kathleen, Schoonjans, Luc, Carmeliet, Peter, Galkin, Alexander, Ichinose, Fumito, Zapol, Warren M., Mootha, Vamsi K.
Format: Artigo
Idioma:Inglês
Publicat: 2019
Matèries:
Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC6903907/
https://ncbi.nlm.nih.gov/pubmed/31402314
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.cmet.2019.07.006
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