Alteration of Cardiolipin Biosynthesis and Remodeling in Single Right Ventricle Congenital Heart Disease.

Alteration of Cardiolipin Biosynthesis and Remodeling in Single Right Ventricle Congenital Heart Disease. Am J Physiol Heart Circ Physiol. 2020 Feb 14;: Authors: Garcia AM, McPhaul JC, Sparagna GC, Jeffrey DA, Jonscher RL, Patel S, Sucharov CC, Stauffer BL, Miyamoto SD, Chatfield KC Abstract Despite advances in both medical and surgical therapies, individuals with single ventricle heart disease (SV) remain at high risk for the development of heart failure (HF). However, the molecular mechanisms underlying remodeling and eventual HF in SV patients are poorly characterized. Cardiolipin (CL), an inner mitochondrial membrane phospholipid, is critical for proper mitochondrial function, and abnormalities in CL content and composition are known in various cardiovascular disease etiologies. The purpose of this study was to investigate myocardial CL content and composition in failing and non-failing single right ventricle (RV) samples compared to normal control RV samples, assess mRNA expression of CL biosynthetic and remodeling enzymes, and quantitate relative mitochondrial copy number. A cross-sectional analysis of RV myocardial tissue from 22 failing SV (SVHF), 9 non-failing SV (SVNF), and 10 bi-ventricular control samples (BVNF) was performed. Expression of enzymes involved in CL biosynthesis and remodeling were analyzed using RT-qPCR and relative mitochondrial DNA copy number determined by qPCR. Normal phase high-pressure liquid chromato...
Source: American Journal of Physiology. Heart and Circulatory Physiology - Category: Physiology Authors: Tags: Am J Physiol Heart Circ Physiol Source Type: research