Because striated muscle mitochondria are uniquely enriched for the remodeled CL species (C18:2)4, mitochondriaare severely impacted by TAZ deficiency, and BTHS is predominately characterized by cardiomyopathy and hypotonia [7,8,9,10,11,12], we focused on examining the effects of acyltransferase deficiency in surviving TazPM♂ hearts. Here, TAFAZZIN is linked to cardiomyopathy.