Supplement prevented mitochondrial problems in diseased mice
An eight-week experiment in mice modeling an inherited muscle disease tested nicotinamide riboside, which prevented dysfunction in the cell structures that produce energy.
Our read
Interventions- Studied:
- Experiments in mice modeling nemaline myopathy type 6, eight weeks
- What changed:
- Nicotinamide riboside prevented the onset of mitochondrial dysfunction in young disease-model mice
- The catch:
- Treatment began before dysfunction; reversal of existing mitochondrial problems was not measured
- Stake:
- Novo Nordisk funded the study.
- In the record:
- One of 601 papers on nicotinamide riboside on record: 112 with results in people, 246 in animals.
- Why it’s aging research:
- It studies an intervention with evidence of extending healthy life.
Editor of record: Boris Djordjevic · AgingResearch.News
Male and female mice engineered with two copies of a gene variant that causes nemaline myopathy type 6, a congenital muscle disease, were studied in an animal experiment. Researchers gave nicotinamide riboside in drinking water at 500 milligrams per kilogram of body weight daily for eight weeks, starting at four weeks old.
The supplement provides a building block for nicotinamide adenine dinucleotide, a molecule involved in energy metabolism. Disease-model mice had lower levels of this molecule than unaffected mice throughout disease progression. Mitochondria are the cell structures that produce energy. The authors concluded that supplementation begun before symptoms appeared prevented the onset of mitochondrial dysfunction in these young disease-model mice. However, supplementation did not prevent abnormal areas called cores from forming in muscle fibers.
Why it matters
The work addressed when mitochondrial problems emerge as an inherited muscle disease progresses with age. The authors described the findings as proof of concept for nicotinamide riboside as a therapeutic strategy for that disease.
Caveats
This prevention experiment began before mitochondrial dysfunction appeared; it did not test reversal of established dysfunction. The findings came from mice with an inherited muscle disease, not people or a study of normal aging.
- Nicotinamide riboside
- KBTBD13
- NAD+ metabolism
- Nemaline myopathy type 6
- Metabolic treadmill performance
- Mice
The paper
Nicotinamide riboside prevents mitochondrial dysfunction in nemaline myopathy type 6
Amsterdam Cardiovascular Sciences
Human Molecular Genetics, Jan 1, 2026, Open access

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