Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease

Cysteamine bitartrate is a US Food and Drug Administration-approved therapy for nephropathic cystinosis also postulated to enhance glutathione biosynthesis. We hypothesized this antioxidant effect may reduce oxidative stress in primary mitochondrial respiratory chain (RC) disease, improving cellular...

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Détails bibliographiques
Publié dans:Hum Mol Genet
Auteurs principaux: Guha, Sujay, Konkwo, Chigoziri, Lavorato, Manuela, Mathew, Neal D, Peng, Min, Ostrovsky, Julian, Kwon, Young-Joon, Polyak, Erzsebet, Lightfoot, Richard, Seiler, Christoph, Xiao, Rui, Bennett, Michael, Zhang, Zhe, Nakamaru-Ogiso, Eiko, Falk, Marni J
Format: Artigo
Langue:Inglês
Publié: Oxford University Press 2019
Sujets:
General Article
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC6522065/
https://ncbi.nlm.nih.gov/pubmed/30668749
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddz023
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