TY - JOUR AU - Martinez-Zamora, Ana AU - Meseguer, Salvador AU - Esteve, Juan M. AU - Villarroya, Magda AU - Aguado, Carmen AU - Enriquez, Jose Antonio AU - Knecht, Erwin AU - Armengod, M. -Eugenia PY - 2015 DO - 10.1371/journal.pone.0144273 SN - 1932-6203 UR - http://hdl.handle.net/20.500.12105/5253 AB - 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... LA - eng PB - Public Library of Science (PLOS) KW - RESPIRATORY-CHAIN DEFICIENCY KW - FATTY-ACID OXIDATION KW - ESCHERICHIA-COLI KW - LACTIC-ACIDOSIS KW - HYPERTROPHIC CARDIOMYOPATHY KW - PHYSIOLOGICALLY RELEVANT KW - GLUTAMINE OXIDATION KW - H2O2 GENERATION KW - ROS PRODUCTION KW - CELL-SURVIVAL TI - 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 TY - journal article ER -