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Patients with Barth syndrome are often affected by severe exercise intolerance, which reduces their overall quality of life. For the first time, we show that nicotinamide riboside can restore exercise tolerance and mitochondrial respiration in a Drosophila Tafazzin mutant, and that the beneficial results are dependent on sir2 and spargel. mutants with overexpressing spargel had a greater number of CL in mutants. In addition, muscle and neurons were identified as key targets for future therapy because sir2 or spargel overexpression in either of these tissues is sufficient to restore the exercise capacity of Drosophila Tafazzin mutants.
Source link: https://europepmc.org/article/MED/36107830
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