TAZ encodes tafazzin, a transacylase essential for cardiolipin formation and central to the etiology of Barth syndrome

•TAZ encodes the tafazzin transacylase responsible for cardiolipin (CL) remodeling.•Tafazzin localizes to the mitochondrial membranes for direct access to CL.•Tafazzin generates mature CL to maintain mitochondrial structure-function.•Mutations throughout TAZ cause the rare mitochondrial disease Barth syndrome.•There is currently no known cure for Barth syndrome.•Potential treatments: PPAR agonists, AAV9 gene therapy, and CL protection.