The Function of Tafazzin, a Mitochondrial Phospholipid–Lysophospholipid Acyltransferase. Issue 18 (21st August 2020)

• Record Type: Journal Article
• Authors: Schlame, Michael; Xu, Yang
• Is Part Of: Journal of molecular biology. Volume 432:Issue 18(2020)
• Page Start: 5043
• Page End: 5051
• Publication Date: 2020-08-21
• Subjects: cardiolipin -- mitochondria -- Barth syndrome -- membrane lipids; CL cardiolipin -- MLCL monolyso-cardiolipin -- PC phosphatidylcholine; Molecular biology -- Periodicals; Biology -- Periodicals; Biochemistry -- Periodicals; Bacteriology -- Periodicals; Molecular Biology -- Periodicals; Biochemistry -- Periodicals; Biologie moléculaire -- Périodiques; Biologie -- Périodiques; Biochimie -- Périodiques; Moleculaire biologie; Biochemistry; Biology; Molecular biology; Periodicals; 572.805
• DOI: 10.1016/j.jmb.2020.03.026
• Languages: English
• ISSNs: 0022-2836

Abstract

Abstract: 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. Graphical abstract: Unlabelled Image Highlights: Tafazzin is a mitochondrial enzyme. It exchanges fatty acids between phospholipids and lysophospholipids. The reaction alters the molecular species composition of membrane lipids. The most important substrate of tafazzin cardiolipin. Tafazzin mutations cause the human disease Barth syndrome.