The enzymatic function of tafazzin

J Biol Chem. 2006 Dec 22;281(51):39217-24. doi: 10.1074/jbc.M606100200. Epub 2006 Nov 2.


Tafazzin is a putative enzyme that is involved in cardiolipin metabolism, it may carry mutations responsible for Barth syndrome. To identify the biochemical reaction catalyzed by tafazzin, we expressed the full-length isoform of Drosophila melanogaster tafazzin in a baculovirus-Sf9 insect cell system. Tafazzin expression induced a new enzymatic function in Sf9 cell mitochondria, namely 1-palmitoyl-2-[14C]linoleoyl-phosphatidylcholine:monolysocardiolipin linoleoyltransferase. We also found evidence for the reverse reaction, because tafazzin expression caused transfer of acyl groups from phospholipids to 1-[14C]palmitoyl-2-lyso-phosphatidylcholine. An affinity-purified tafazzin construct, tagged with the maltose-binding protein, catalyzed both forward and reverse transacylations between cardiolipin and phosphatidylcholine, but was unable to utilize CoA or acyl-CoA as substrates. Whereas tafazzin supported transacylations between various phospholipid-lysophospholipid pairs, it showed the highest rate for the phosphatidylcholine-cardiolipin transacylation. Transacylation activities were about 10-fold higher for linoleoyl groups than for oleoyl groups, and they were negligible for arachidonoyl groups. The data show that Drosophila tafazzin is a CoA-independent, acyl-specific phospholipid transacylase with substrate preference for cardiolipin and phosphatidylcholine.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • 1-Acylglycerophosphocholine O-Acyltransferase / chemistry
  • 1-Acylglycerophosphocholine O-Acyltransferase / physiology*
  • Acylation
  • Animals
  • Baculoviridae / metabolism
  • Cardiolipins / metabolism
  • Cell Line
  • Drosophila Proteins / chemistry
  • Drosophila Proteins / physiology*
  • Gene Expression Regulation, Enzymologic*
  • Insecta
  • Mitochondria / metabolism
  • Models, Chemical
  • Phosphatidylcholines / chemistry
  • Phospholipids / chemistry
  • Protein Binding
  • Substrate Specificity
  • Time Factors


  • Cardiolipins
  • Drosophila Proteins
  • Phosphatidylcholines
  • Phospholipids
  • 1-Acylglycerophosphocholine O-Acyltransferase
  • tafazzin protein, Drosophila