作者: Grant R. Macgregor , Douglas C. Wallace , Brett C. Graham
DOI:
关键词: Biochemistry 、 Genetically modified mouse 、 Oxidative phosphorylation 、 Biology 、 Mitochondrial myopathy 、 Adenine nucleotide translocator 、 Hypertrophic cardiomyopathy 、 Gene isoform 、 Inner mitochondrial membrane 、 Animal 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.