Despite the known importance of mitochondrial function and CL remodeling in BTHS as well as in cardiovascular health within the general population [13], how TAZ mutations cause the variety of divergent and fluctuating heart phenotypes seen in BTHS patients is poorly understood [4,8,14] This stems from an incomplete understanding of BTHS-affected downstream signaling, inconsistent genotype-phenotype correlations, and a lack of patient-tailored mouse models to interrogate mutation-specific effects [9,15]. Here, TAFAZZIN is linked to Barth syndrome.