As mitochondrial defects are a hallmark of BTHS [3, 5] and as TAZ itself impacts many aspects of mitochondrial structure and function [1, 39, 40], we examined the mitochondrial content and membrane potential (Δψm) across HSC and HPC populations in TazD75H♂ and wt♂ mice. Here, TAFAZZIN is linked to Barth syndrome.