Niacin Cures Systemic NAD(+) Deficiency and Improves Muscle Performance in Adult-Onset Mitochondrial Myopathy

Show full item record



Permalink

http://hdl.handle.net/10138/330502

Citation

Pirinen , E , Auranen , M , Khan , N A , Brilhante , V , Urho , N , Pessia , A , Hakkarainen , A , Kuula , J , Heinonen , U , Schmidt , M S , Haimilahti , K , Piirilä , P , Lundbom , N , Taskinen , M-R , Brenner , C , Velagapudi , V , Pietiläinen , K H & Suomalainen , A 2020 , ' Niacin Cures Systemic NAD(+) Deficiency and Improves Muscle Performance in Adult-Onset Mitochondrial Myopathy ' , Cell Metabolism , vol. 31 , no. 6 , pp. 1078-1090 . https://doi.org/10.1016/j.cmet.2020.04.008

Title: Niacin Cures Systemic NAD(+) Deficiency and Improves Muscle Performance in Adult-Onset Mitochondrial Myopathy
Author: Pirinen, Eija; Auranen, Mari; Khan, Nahid A.; Brilhante, Virginia; Urho, Niina; Pessia, Alberto; Hakkarainen, Antti; Kuula, Juho; Heinonen, Ulla; Schmidt, Mark S.; Haimilahti, Kimmo; Piirilä, Päivi; Lundbom, Nina; Taskinen, Marja-Riitta; Brenner, Charles; Velagapudi, Vidya; Pietiläinen, Kirsi H.; Suomalainen, Anu
Contributor: University of Helsinki, CAMM - Research Program for Clinical and Molecular Metabolism
University of Helsinki, HUS Neurocenter
University of Helsinki, STEMM - Stem Cells and Metabolism Research Program
University of Helsinki, University of Helsinki
University of Helsinki, Institute for Molecular Medicine Finland
University of Helsinki, HUS Medical Imaging Center
University of Helsinki, HUS Medical Imaging Center
University of Helsinki, Research Programs Unit
University of Helsinki, HUS Medical Imaging Center
University of Helsinki, HUS Medical Imaging Center
University of Helsinki, HUS Heart and Lung Center
University of Helsinki, Institute for Molecular Medicine Finland
University of Helsinki, HUS Abdominal Center
University of Helsinki, HUSLAB
Date: 2020-06-02
Language: eng
Number of pages: 18
Belongs to series: Cell Metabolism
ISSN: 1550-4131
URI: http://hdl.handle.net/10138/330502
Abstract: 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.
Subject: FATTY LIVER-DISEASE
NICOTINAMIDE RIBOSIDE
MULTIPLE DELETIONS
LIFE-SPAN
DNA
SPECTROSCOPY
ADIPONECTIN
METABOLISM
MUTATIONS
BIOMARKER
1182 Biochemistry, cell and molecular biology
3112 Neurosciences
3124 Neurology and psychiatry
Rights:


Files in this item

Total number of downloads: Loading...

Files Size Format View
Pirinen_all_Cell_Metab_final_070620.pdf 328.7Kb PDF View/Open

This item appears in the following Collection(s)

Show full item record