Defective Expression of the Mitochondrial-tRNA Modifying Enzyme GTPBP3 Triggers AMPK-Mediated Adaptive Responses Involving Complex I Assembly Factors, Uncoupling Protein 2, and the Mitochondrial Pyruvate Carrier

GTPBP3 is an evolutionary conserved protein presumably involved in mitochondrial tRNA (mt-tRNA) modification. In humans, GTPBP3 mutations cause hypertrophic cardiomyopathy with lactic acidosis, and have been associated with a defect in mitochondrial translation, yet the pathomechanism remains unclea...

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Detalles Bibliográficos
Autores principales: Martínez-Zamora, Ana, Meseguer, Salvador, Esteve, Juan M., Villarroya, Magda, Aguado, Carmen, Enríquez, J. Antonio, Knecht, Erwin, Armengod, M.-Eugenia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2015
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4671719/
https://www.ncbi.nlm.nih.gov/pubmed/26642043
http://dx.doi.org/10.1371/journal.pone.0144273

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4671719/
https://www.ncbi.nlm.nih.gov/pubmed/26642043
http://dx.doi.org/10.1371/journal.pone.0144273

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