One of the most studied illnesses is Barth syndrome (BTHS), an X-linked pathology caused by a mutation of the Tafazzin gene (TAZ), which encodes a mitochondrial cardiolipin remodeling/maturation acyltransferase [97]; the deacylation of nascent cardiolipin leads to monolysocardiolipin accumulation [96]. The gene discussed is TAFAZZIN; the disease is Barth syndrome.