Article
Cardiolipin Remodeling Defects Impair Mitochondrial Architecture and Function in a Murine Model of Barth Syndrome Cardiomyopathy.
Circulation. Heart failure - 1 Jun 2021
Zhu Siting, Chen Ze'e, Zhu Mason, Shen Ying, Leon Leonardo J, Chi Liguo, Spinozzi Simone, Tan Changming, Gu Yusu, Nguyen Anh, Zhou Yi, Feng Wei, Vaz Frédéric M, Wang Xiaohong, Gustafsson Asa B, Evans Sylvia M, Kunfu Ouyang, Fang Xi
Abstract excerpt
BACKGROUND: Cardiomyopathy is a major clinical feature in Barth syndrome (BTHS), an X-linked mitochondrial lipid disorder caused by mutations in Tafazzin (TAZ), encoding a mitochondrial acyltransferase required for cardiolipin remodeling. Despite recent description of a mouse model of BTHS cardiomyopathy, an in-depth analysis of specific lipid abnormalities and mitochondrial form and function in an in vivo BTHS...
Topics
Join the communities discussing this publication.