Mouse lacking heart-muscle adenine nucleotide translocator protein and methods

作者: Grant R. Macgregor , Douglas C. Wallace , Brett C. Graham

DOI:

关键词: BiochemistryGenetically modified mouseOxidative phosphorylationBiologyMitochondrial myopathyAdenine nucleotide translocatorHypertrophic cardiomyopathyGene isoformInner mitochondrial membraneAnimal model

摘要: Provided are transgenic mice genetically engineered for a deficiency of the heart-skeletal muscle isoform adenine nucleotide translocator protein (Ant1). These exhibit histological, biochemical, and physiological signs in oxidative phosphorylation energy generation, these provide first animal model mitochondrial myopathy hypertrophic cardiomyopathy. This is used methods testing compounds therapeutic value treating failure to exchange ATP ADP across inner membrane, OXPHOS cardiac hypertrophy.

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