Barth syndrome is an X-linked genetic disorder caused by mutations in the tafazzin (taz)
gene and characterized by dilated cardiomyopathy, exercise intolerance, chronic fatigue,
delayed growth, and neutropenia. Tafazzin is a mitochondrial transacylase required for
cardiolipin remodeling. Although tafazzin function has been studied in non-mammalian
model organisms, mammalian genetic loss of function approaches have not been used. We
examined the consequences of tafazzin knockdown on sarcomeric mitochondria and …