Barth syndrome (BTHS) is an X-linked mitochondrial lipid disorder caused by mutations in
the TAFAZZIN (TAZ) gene, which encodes a mitochondrial acyltransferase/transacylase …

It is now well known that oxidative stress promotes lipid peroxidation, protein oxidation,
activation of proteases, fragmentation of DNA and alteration in gene expression for …

Mitochondria are paramount to the metabolism and survival of cardiomyocytes. Here we
show that Mitochondrial Fission Process 1 (MTFP1) is an inner mitochondrial membrane …

Electrophysiological and structural disruptions in cardiac arrhythmias are closely related to
mitochondrial dysfunction. Mitochondria are an organelle generating ATP, thereby satisfying …

Cardiolipin (CL) is a unique, tetra-acylated diphosphatidylglycerol lipid that mainly localizes
in the inner mitochondria membrane (IMM) in mammalian cells and plays a central role in …

Barth Syndrome (BTHS) is an inherited cardiomyopathy caused by defects in the
mitochondrial transacylase TAFAZZIN (Taz), required for the synthesis of the phospholipid …

Barth syndrome is an X-linked disorder caused by loss-of-function mutations in Tafazzin
(TAZ), an acyltransferase that catalyzes remodeling of cardiolipin, a signature phospholipid …

Abstract Purpose of Review We review pathophysiology and clinical features of
mitochondrial disorders manifesting with cardiomyopathy. Recent Findings Mechanistic …

Barth syndrome (BTHS) is a debilitating X-linked cardio-skeletal myopathy caused by loss-of-
function mutations in TAFAZZIN, a cardiolipin (CL)-remodeling enzyme required for the …

Introduction Abnormal calcium signaling between organelles such as the sarcoplasmic
reticulum (SR), mitochondria and lysosomes is a key feature of heart diseases. Calcium …