Defects in the mitochondrial-tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARγ-UCP2-AMPK axis

Human proteins MTO1 and GTPBP3 are thought to jointly catalyze the modification of the wobble uridine in mitochondrial tRNAs. Defects in each protein cause infantile hypertrophic cardiomyopathy with lactic acidosis. However, the underlying mechanisms are mostly unknown. Using fibroblasts from an MTO...

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Detalles Bibliográficos
Autores principales: Boutoual, Rachid, Meseguer, Salvador, Villarroya, Magda, Martín-Hernández, Elena, Errami, Mohammed, Martín, Miguel A., Casado, Marta, Armengod, M.-Eugenia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2018
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5773609/
https://www.ncbi.nlm.nih.gov/pubmed/29348686
http://dx.doi.org/10.1038/s41598-018-19587-5

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5773609/
https://www.ncbi.nlm.nih.gov/pubmed/29348686
http://dx.doi.org/10.1038/s41598-018-19587-5

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