TAFAZZIN and Barth syndrome: Thus, while previous transgenic mouse models either lack [8,18,19] or express diminished wild-type Taz protein [20] or exhibit independent doxycycline-mediated OxPhos suppression [46], our TazPM allele provides, to the best of our knowledge, the first animal model that faithfully recapitulates the genomic origin of BTHS and serves as the first in vivo BTHS model expressing a mutant protein.