Circ Heart Fail 2021 Jun 15;14(6):e008289. Epub 2021 Jun 15.
Department of Medicine (S.Z., Z.C., M.Z., S.S., C.T., Y.G., A.N., W.F., S.M.E., X.F.), University of California, San Diego, La Jolla.
Background: Cardiomyopathy is a major clinical feature in Barth syndrome (BTHS), an X-linked mitochondrial lipid disorder caused by mutations in (), 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 cardiomyopathy model is lacking.
Methods: We performed in-depth assessment of cardiac function, cardiolipin species profiles, and mitochondrial structure and function in our newly generated cardiomyocyte-specific knockout mice and Cre-negative control mice (n≥3 per group). Read More