Tafazzin is a mitochondrial enzyme that exchanges fatty acids between phospholipids by phospholipid–lysophospholipid transacylation. The reaction alters the molecular species composition and, as a result, the physical properties of lipids. In vivo, the most important substrate of tafazzin is the mitochondria-specific lipid cardiolipin. Tafazzin mutations cause the human disease Barth syndrome, which presents with cardiomyopathy, skeletal muscle weakness, fatigue, and other symptoms, probably all related to mitochondrial dysfunction. The reason why mitochondria require tafazzin is still not known, but recent evidence suggests that tafazzin may lower the energy cost associated with protein crowding in the inner mitochondrial membrane.

Tafazzin 是一种线粒体酶，通过磷脂-溶血磷脂转酰基作用在磷脂之间交换脂肪酸。该反应改变了分子种类的组成，从而改变了脂质的物理特性。在体内，tafazzin 最重要的底物是线粒体特异性脂质心磷脂。Tafazzin 突变导致人类疾病 Barth 综合征，表现为心肌病、骨骼肌无力、疲劳和其他症状，可能都与线粒体功能障碍有关。线粒体需要 tafazzin 的原因尚不清楚，但最近的证据表明，tafazzin 可能会降低与线粒体内膜中蛋白质拥挤相关的能量成本。