Nicotinamide Riboside Improves Mitochondrial Function and Exercise Ability in ANT1-Deficiency: Preclinical Findings

Synopsis

Mitochondrial disorders are often characterized by muscle weakness and fatigue. Mutations in the heart-muscle adenine nucleotide translocator isoform 1 (ANT1) (or ANT1-deficiency) of both humans and mice cause muscle weakness and exercise intolerance linked to impaired mitochondrial respiration and NAD+ depletion. This study showed that ANT1-deficient mice had reduced complex I respiration and TCA cycle stalling during exercise due to lack of NAD+. Nicotinamide riboside supplementation raised NAD+ levels, improved mitochondrial function, and increased exercise capacity. This indicates NAD+ boosters like NR could treat mitochondrial myopathies caused by ANT1 defects.

Journal

Molecular Metabolism