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Myocardial disturbances of intermediary metabolism in Barth syndrome

Barth Syndrome (BTHS) is a rare X-linked mitochondrial disorder due to mutations in the gene TAFAZZIN, which leads to immature cardiolipin (CL) remodeling and is characterized by the development of cardiomyopathy. The immature CL remodeling in BTHS results in electron transport chain respiratory def...

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Detalles Bibliográficos
Autores principales: Greenwell, Amanda A., Tabatabaei Dakhili, Seyed Amirhossein, Ussher, John R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9399503/
https://www.ncbi.nlm.nih.gov/pubmed/36035919
http://dx.doi.org/10.3389/fcvm.2022.981972