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Article

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

Biochimica et biophysica acta - 1 Jun 2012

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

Abstract excerpt

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-threatening neurological syndrome known as MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like...

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Identifiers

PMID: 22306605
DOI: 10.1016/j.bbadis.2012.01.010
PMCID: PMC3339237

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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