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

Boutoual, R; Meseguer, S; Villarroya, M; Martin-Hernandez, E; Errami, M; Martin, MA; Casado, M; Armengod, ME

Meseguer, S; Armengod, ME (reprint author), CIPF, RNA Modificat & Mitochondrial Dis Lab, Valencia 46012, Spain.; Armengod, ME (reprint author), Ctr Invest Biomed Red Enfermedades Raras CIBERER, Node 721, Madrid 28029, Spain.

SCIENTIFIC REPORTS, 2018; 8 ():

Abstract

Human proteins MTO1 and GTPBP3 are thought to jointly catalyze the modification of the wobble uridine in mitochondrial tRNAs. Defects in each protein ......

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