Metabolically induced heteroplasmy shifting and L-arginine treatment reduce the energetic defect in a neuronal-like model of MELAS

The m.3243A>G variant in the mitochondrial tRNA(Leu (UUR)) gene is a common mitochondrial DNA (mtDNA) mutation. Phenotypic manifestations depend mainly on the heteroplasmy, i.e. the ratio of mutant to normal mtDNA copies. A high percentage of mutant mtDNA is associated with a severe, life-threate...

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Main Authors: Desquiret-Dumas, Valerie, Gueguen, Naig, Barth, Magalie, Chevrollier, Arnaud, Hancock, Saege, Wallace, Douglas C, Amati-Bonneau, Patrizia, Henrion, Daniel, Bonneau, Dominique, Reynier, Pascal, Procaccio, Vincent
Format: Artigo
Language:Inglês
Published: 2012
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Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC3339237/
https://ncbi.nlm.nih.gov/pubmed/22306605
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.bbadis.2012.01.010
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