Mouse lacking heart-muscle adenine nucleotide translocator prote

Multicellular living organisms and unmodified parts thereof and – Nonhuman animal – Transgenic nonhuman animal

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800 3, 800 9, 800 21, 800 22, 800 25, 435455, 435463, 4353201, 435325, C12N 1509, C12N 1563, C12N 1500, C12N 500

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060138580

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

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