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Article

Measurement of ATP production in mitochondrial disorders.

Journal of inherited metabolic disease - 1 Feb 2006

Shepherd R K, Checcarelli N, Naini A, De Vivo D C, DiMauro S, Sue C M

Abstract excerpt

Mitochondrial diseases are a heterogeneous group of disorders caused by mutations in both nuclear DNA (nDNA) and mitochondrial DNA (mtDNA). Mitochondrial disease leads to impaired respiratory chain function and reduced ATP production. The aim of this study was to compare disturbances in mitochondrial function by measuring ATP synthesis in fibroblasts derived from patients with nDNA and mtDNA defects. Skin...

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PMID: 16601873
DOI: 10.1007/s10545-006-0148-8

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