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Stimulating myocardial pyruvate dehydrogenase activity fails to alleviate cardiac abnormalities in a mouse model of human Barth syndrome

Barth syndrome (BTHS) is a rare genetic disorder due to mutations in the TAFAZZIN gene, leading to impaired maturation of cardiolipin and thereby adversely affecting mitochondrial function and energy metabolism, often resulting in cardiomyopathy. In a murine model of BTHS involving short-hairpin RNA...

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Detalles Bibliográficos
Autores principales: Greenwell, Amanda A., Tabatabaei Dakhili, Seyed Amirhossein, Gopal, Keshav, Saed, Christina T., Chan, Jordan S. F., Kazungu Mugabo, Nick, Zhabyeyev, Pavel, Eaton, Farah, Kruger, Jennifer, Oudit, Gavin Y., 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/PMC9537754/
https://www.ncbi.nlm.nih.gov/pubmed/36211560
http://dx.doi.org/10.3389/fcvm.2022.997352