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Barth Syndrome - Europe PMC

Summarized by Plex Scholar
Last Updated: 10 October 2022

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Stimulating the sir2-spargel axis rescues exercise capacity and mitochondrial respiration in a Drosophila model of Barth syndrome.

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

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions