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Article

Respiratory-deficient human fibroblasts exhibiting defective mitochondrial DNA replication.

The Biochemical journal - 1 Feb 1995

Bodnar A G, Cooper J M, Leonard J V, Schapira A H

Abstract excerpt

We have characterized cultured skin fibroblasts from two siblings affected with a fatal mitochondrial disease caused by a nuclear genetic defect. Mitochondrial respiratory-chain function was severely decreased in these cells. Southern-blot analysis showed that the fibroblasts had reduced levels of mitochondrial DNA (mtDNA). The mtDNA was unstable and was eliminated from the cultured cells over many generations,...

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Identifiers

PMID: 7848281
DOI: 10.1042/bj3050817
PMCID: PMC1136332

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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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