TAFAZZIN and Barth syndrome: In the TAZ pathway, newly synthesized cardiolipin is proposed to be deacylated and reacylated by TAZ. It appears that this mechanism is essential for optimal mitochondrial function in heart because Barth Syndrome, which is characterized by a severe cardiomyopathy [95, 96], is caused by a mutated TAZ gene that encodes a putative mitochondrial phospholipid acyltransferase with both deacylation and reacylation activities [95, 97].