nicotinamide-beta-riboside and Muscle-Weakness

Mitochondrial disorders are often characterized by muscle weakness and fatigue. Null mutations in the heart-muscle adenine nucleotide translocator isoform 1 (ANT1) of both humans and mice cause cardiomyopathy and myopathy associated with exercise intolerance and muscle weakness. Here we decipher the molecular underpinnings of ANT1-deficiency-mediated exercise intolerance.. This was achieved by correlating exercise physiology, mitochondrial function and metabolomics of mice deficient in ANT1 and comparing this to control mice.. We demonstrate a peripheral limitation of skeletal muscle mitochondrial respiration and a reduced complex I respiration in ANT1-deficient mice. Upon exercise, this results in a lack of NAD. Increasing NAD

Topics: Adenine Nucleotide Translocator 1; Animals; Mice; Mitochondrial Myopathies; Muscle Weakness; NAD; Niacinamide; Physical Conditioning, Animal; Protein Isoforms; Pyridinium Compounds