期刊
CELL METABOLISM
卷 31, 期 6, 页码 1078-+出版社
CELL PRESS
DOI: 10.1016/j.cmet.2020.04.008
关键词
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资金
- Centre of Excellence from Academy of Finland [307431]
- academy grants [307592, 303349, 286359, 272376, 314383, 266286]
- Sigrid Juselius Foundation
- United Mitochondrial Disease Foundation
- Finnish Medical Foundation
- Novo Nordisk Foundation
- Gyllenberg Foundation
- Finnish Diabetes Research Foundation
- Finnish Foundation for Cardiovascular Research
- HiLife/University of Helsinki
- Government Research Funds (Helsinki University Hospital)
- Academy of Finland (AKA) [307431, 307431] Funding Source: Academy of Finland (AKA)
NAD(+) is a redox-active metabolite, the depletion of which has been proposed to promote aging and degenerative diseases in rodents. However, whether NAD(+) depletion occurs in patients with degenerative disorders and whether NAD(+) repletion improves their symptoms has remained open. Here, we report systemic NAD(+) deficiency in adult-onset mitochondrial myopathy patients. We administered an increasing dose of NAD(+) booster niacin, a vitamin B3 form (to 750-1,000 mg/day; clinicaltrials.gov NCT03973203) for patients and their matched controls for 10 or 4 months, respectively. Blood NAD(+) increased in all subjects, up to 8-fold, and muscle-NAD(+) of patients reached the level of their controls. Some patients showed anemia tendency, while muscle strength and mitochondrial biogenesis increased in all subjects. In patients, muscle metabolome shifted toward controls and liver fat decreased even 50%. Our evidence indicates that blood analysis is useful in identifying NAD(+) deficiency and points niacin to be an efficient NAD(+) booster for treating mitochondrial myopathy.
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