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Article

Mitochondrial genome maintenance in health and disease.

DNA repair - 1 Jul 2014

Copeland William C, Longley Matthew J

Abstract excerpt

Human mitochondria harbor an essential, high copy number, 16,569 base pair, circular DNA genome that encodes 13 gene products required for electron transport and oxidative phosphorylation. Mutation of this genome can compromise cellular respiration, ultimately resulting in a variety of progressive metabolic diseases collectively known as 'mitochondrial diseases'. Mutagenesis of mtDNA and the persistence of mtDNA...

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Identifiers

PMID: 24780559
DOI: 10.1016/j.dnarep.2014.03.010
PMCID: PMC4075028

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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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