LCLAT1 and cardiomyopathy: In support of the causative role of ALCAT1 in mitochondrial dysfunction in BTHS, ALCAT1 protein expression in the heart is upregulated by TAZ deficiency, whereas targeted deletion of ALCAT1 not only prevents cardiomyopathy, but also restores mitochondrial morphology and respiration without significant effects on TLCL levels in the hearts of a mouse model of inducible TAZ depletion (Zhang and Shi, unpublished data).