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

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...

Full description

Bibliographic Details
Main Authors: Amanda A. Greenwell, Seyed Amirhossein Tabatabaei Dakhili, Keshav Gopal, Christina T. Saed, Jordan S. F. Chan, Nick Kazungu Mugabo, Pavel Zhabyeyev, Farah Eaton, Jennifer Kruger, Gavin Y. Oudit, John R. Ussher
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-09-01
Series:Frontiers in Cardiovascular Medicine
Subjects:
dichloroacetate
Barth syndrome
cardiomyopathy
pyruvate dehydrogenase
glucose oxidation
Online Access:https://www.frontiersin.org/articles/10.3389/fcvm.2022.997352/full

Internet

https://www.frontiersin.org/articles/10.3389/fcvm.2022.997352/full

Similar Items