The Metabolomic Bioenergetic Signature of Opa1-Disrupted Mouse Embryonic Fibroblasts Highlights Aspartate Deficiency

OPA1 (Optic Atrophy 1) is a multi-isoform dynamin GTPase involved in the regulation of mitochondrial fusion and organization of the cristae structure of the mitochondrial inner membrane. Pathogenic OPA1 variants lead to a large spectrum of disorders associated with visual impairment due to optic ner...

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Udgivet i:Sci Rep
Main Authors: Bocca, Cinzia, Kane, Mariame Selma, Veyrat-Durebex, Charlotte, Chupin, Stéphanie, Alban, Jennifer, Kouassi Nzoughet, Judith, Le Mao, Morgane, Chao de la Barca, Juan Manuel, Amati-Bonneau, Patrizia, Bonneau, Dominique, Procaccio, Vincent, Lenaers, Guy, Simard, Gilles, Chevrollier, Arnaud, Reynier, Pascal
Format: Artigo
Sprog:Inglês
Udgivet: Nature Publishing Group UK 2018
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6070520/
https://ncbi.nlm.nih.gov/pubmed/30068998
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41598-018-29972-9
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