TAFAZZIN and Barth syndrome: As reported, ShTaz1 poorly accumulates Tafazzin, is defective in CL maturation, has a reduced capacity to associate respiratory chain complexes into ‘respirasomes’, produces abnormally enlarged cells and has a higher content in mitochondria compared to shWT1 and shTaz1R, as do cells from BTHS patients 24, 48, 66.