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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...
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. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2022
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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 |
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