Diverse mitochondrial abnormalities in a new cellular model of TAFFAZZIN deficiency are remediated by cardiolipin-interacting small molecules

Barth syndrome (BTHS) is an X-linked disorder of mitochondrial phospholipid metabolism caused by pathogenic variants in TAFFAZIN, which results in abnormal cardiolipin (CL) content in the inner mitochondrial membrane. To identify unappreciated pathways of mitochondrial dysfunction in BTHS, we utiliz...

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Detalles Bibliográficos
Autores principales: Anzmann, Arianna F., Sniezek, Olivia L., Pado, Alexandra, Busa, Veronica, Vaz, Frédéric M., Kreimer, Simion D., DeVine, Lauren R., Cole, Robert N., Le, Anne, Kirsch, Brian J., Claypool, Steven M., Vernon, Hilary J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2021
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8384898/
https://www.ncbi.nlm.nih.gov/pubmed/34314685
http://dx.doi.org/10.1016/j.jbc.2021.101005

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