Article
Genetic background influences mitochondrial function: modeling mitochondrial disease for therapeutic development.
Trends in molecular medicine - 1 May 2010
Bénit Paule, El-Khoury Riyad, Schiff Manuel, Sainsard-Chanet Annie, Rustin Pierre
Abstract excerpt
Genetic background strongly influences the phenotype of human mitochondrial diseases. Mitochondrial biogenesis and function require up to 1500 nuclear genes, providing myriad opportunities for effects on disease expression. Phenotypic variability, combined with relative rarity, constitutes a major obstacle to establish cohorts for clinical trials. Animal models are, therefore, potentially valuable. However,...
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