Effective treatment of mitochondrial myopathy by nicotinamide riboside, a vitamin B3

Nicotinamide riboside, a vitamin B3, is effective to delayed early- and late-stage disease progression, via mitochondrial biogenesis in skeletal muscle and brown adipose tissue, thereby preventing mitochondrial ultrastructure abnormalities and mtDNA deletion formation. NR further stimulated mitochondrial unfolded protein response, suggesting its protective role in mitochondrial disease.