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

Cardiolipin (CL) is a phospholipid required for proper mitochondrial function. Tafazzin remodels CL to create highly unsaturated fatty acid chains. However, when TAFAZZIN is mutated, CL remodeling is impeded, leading to mitochondrial dysfunction and the disease Barth syndrome. Patients with Barth sy...

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Detalles Bibliográficos
Autores principales: Damschroder, Deena, Zapata-Pérez, Rubén, Richardson, Kristin, Vaz, Frédéric M., Houtkooper, Riekelt H., Wessells, Robert
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9558626/
https://www.ncbi.nlm.nih.gov/pubmed/36107830
http://dx.doi.org/10.1242/dmm.049279