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Article

MtDNA and nuclear mutations affecting oxidative phosphorylation: correlating severity of clinical defect with extent of bioenergetic compromise.

Journal of bioenergetics and biomembranes - 1 Jun 1994

Robinson B H

Abstract excerpt

Rates of ATP synthesis were studied in cultured skin fibroblasts treated with digitonin. In fibroblasts from patients with complex I deficiency, complex IV and complex V deficiency rates of ATP synthesis were decreased below the levels found in controls. In mitochondria isolated from cultured lymphoblasts, ATP synthesis was also decreased by 35-50% in cases of Leigh's disease due to complex I, complex IV, or...

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PMID: 8077184
DOI: 10.1007/BF00763102

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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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MtDNA and nuclear mutations affecting oxidative phosphorylation: correlating severity of clinical defect with extent of bioenergetic compromise.PMID 8077184Read ↗

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Comparative biochemical studies in fibroblasts from patients with different forms of Leigh syndrome.Semantic proximity Read ↗

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Structure, functioning, and assembly of the ATP synthase in cells from patients with the T8993G mitochondrial DNA mutation. Comparison with the enzyme in Rho(0) cells completely lacking mtdna.Semantic proximity Read ↗

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