烟酸
肌病
NAD+激酶
线粒体肌病
医学
内科学
线粒体
内分泌学
生物
生物化学
线粒体DNA
酶
基因
作者
Eija Pirinen,Mari Auranen,Nahid Akhtar Khan,Virginia Brilhante,Niina Urho,Alberto Pessia,Antti Hakkarainen,Juho Kuula,Ulla Heinonen,Mark Schmidt,Kimmo Haimilahti,Päivi Piirilä,Nina Lundbom,Marja-Riitta Taskinen,Charles Brenner,Vidya Velagapudi,Kirsi H. Pietiläinen,Anu Suomalainen
出处
期刊:Cell Metabolism
[Elsevier]
日期:2020-06-02
卷期号:31 (6): 1078-1090.e5
被引量:102
标识
DOI:10.1016/j.cmet.2020.04.008
摘要
Summary 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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