Browsing by Subject "NADH"

Mitochondrial myopathies are caused by dysfunction of mitochondria, the powerhouses of the cell. Currently, these diseases are lacking cure. However, it was recently shown that patients with advanced mitochondrial myopathy have systemic NAD+ depletion contributing to the disease pathology. Excitingly, NAD+ repletion with NAD+ precursor vitamin B3 niacin efficiently improved the NAD+ levels. Increase in NAD+ levels translated into improved mitochondrial and muscle function. Hereby, I studied whether early-stage mitochondrial myopathy patients could benefit from niacin treatment. Three patients were recruited. They received 500 mg of niacin per day for 10 months. NAD+ levels and other disease markers were analyzed at several time points during the study. In addition, muscle strength, exercise capacity and plasma metabolomics were studied at the beginning and end of the study. Results were compared to the previous study with patients with advanced mitochondrial myopathy and control populations. Niacin efficiently increased blood NAD+ levels in patients. Increase was comparable to published results with 1000 mg of niacin per day. Niacin did not improve the already excellent fitness level of patients. Interestingly, patient metabolomic profile differed from controls and was moved closer to controls with niacin treatment. In conclusion, already 500 mg of niacin seems to be equally efficient to 1000 mg in increasing blood NAD+ levels. This suggest that 500 mg could be enough to treat the patients and thereby some of the adverse effects of niacin, such as cutaneous flushing and possibly anemia, could perhaps be prevented. In addition, metabolic changes seem to precede the development of muscle weakness and metabolic changes were reversed with niacin treatment. Thus, this data suggests early-stage mitochondrial myopathy patients to benefit from niacin treatment.