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A model for determining cardiac mitochondrial substrate utilisation using stable 13 C-labelled metabolites
Ross T. Lindsay, Demetris Demetriou, Dominic Manetta-Jones, James A. West, Andrew J. Murray, Julian L. Griffin
Nov 25, 2020
Abstract: Introduction: Relative oxidation of different metabolic substrates in the heart varies both physiologically and pathologically, in order to meet metabolic demands under different circumstances. 13C labelled substrates...
Dominant ACO2 mutations are a frequent cause of isolated optic atrophy.
Majida Charif, Naïg Gueguen, Marc Ferré, Zouhair Elkarhat, Salim Khiati, Morgane LeMao, Arnaud Chevrollier, Valerie Desquiret-Dumas, David Goudenège, Céline Bris, Selma Kane, Jennifer Alban, Stéphanie Chupin, Céline Wetterwald, Leonardo Caporali, Francesca Tagliavini, Chiara LaMorgia, Michele Carbonelli, Neringa Jurkute, Abdelhamid Barakat, Philippe Gohier, Christophe Verny, Magalie Barth, Vincent Procaccio, Dominique Bonneau, Xavier Zanlonghi, Isabelle Meunier, Nicole Weisschuh, Simone Schimpf-Linzenbold, Felix Tonagel, Ulrich Kellner, Patrick Yu-Wai-Man, Valerio Carelli, Bernd Wissinger, Patrizia Amati-Bonneau, Pascal Reynier, European ION Group, Guy Lenaers
Jul 02, 2021
Biallelic mutations in ACO2, encoding the mitochondrial aconitase 2, have been identified in individuals with neurodegenerative syndromes, including infantile cerebellar retinal degeneration and recessive optic neuropathies...
A model for determining cardiac mitochondrial substrate utilisation using stable 13C-labelled metabolites.
Ross T Lindsay, Demetris Demetriou, Dominic Manetta-Jones, James A West, Andrew J Murray, Julian L Griffin
Nov 21, 2019
A model for determining cardiac mitochondrial substrate utilisation using stable 13C-labelled metabolites.
Ross T Lindsay, Demetris Demetriou, Dominic Manetta-Jones, James A West, Andrew J Murray, Julian L Griffin
Jan 03, 2020
INTRODUCTION:Relative oxidation of different metabolic substrates in the heart varies both physiologically and pathologically, in order to meet metabolic demands under different circumstances. 13C labelled substrates have become...