The Function of Tafazzin, a Mitochondrial Phospholipid-Lysophospholipid Acyltransferase

J Mol Biol. 2020 Aug 21;432(18):5043-5051. doi: 10.1016/j.jmb.2020.03.026. Epub 2020 Mar 29.


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.

Keywords: Barth syndrome; cardiolipin; membrane lipids; mitochondria.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Acyltransferases
  • Animals
  • Barth Syndrome / genetics*
  • Cardiolipins / metabolism
  • Humans
  • Mitochondria / enzymology*
  • Mitochondria / metabolism
  • Mutation
  • Transcription Factors / genetics
  • Transcription Factors / metabolism*


  • Cardiolipins
  • Transcription Factors
  • Acyltransferases
  • TAFAZZIN protein, human