Identification and characterization of human cardiolipin synthase

FEBS Lett. 2006 May 29;580(13):3059-64. doi: 10.1016/j.febslet.2006.04.054. Epub 2006 Apr 27.


The mitochondrial phospholipid cardiolipin is synthesized from cytidinediphosphate-diacylglycerol and phosphatidylglycerol, a process catalyzed by the enzyme cardiolipin synthase. In this study, we identified a human candidate gene/cDNA for cardiolipin synthase, C20orf155. Expression of this candidate cDNA in the (cardiolipin synthase-deficient) crd1Delta yeast confirmed that it indeed encodes human cardiolipin synthase. Purified mitochondria of the crd1Delta expressing human cardiolipin synthase were used to characterize the enzyme. It has an alkaline pH optimum, requires divalent cations for activity and appears to have a different substrate preference for cytidinediphosphate-diacylglycerol species when compared to phosphatidylglycerol species. The possible implications for CL synthesis and remodeling are discussed.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cardiolipins / biosynthesis
  • Cations, Divalent / chemistry
  • Cytidine Diphosphate Diglycerides / chemistry
  • Genetic Complementation Test
  • Humans
  • Hydrogen-Ion Concentration
  • Membrane Proteins / chemistry*
  • Membrane Proteins / genetics*
  • Membrane Proteins / isolation & purification
  • Mitochondria / enzymology
  • Saccharomyces cerevisiae / enzymology
  • Saccharomyces cerevisiae / genetics
  • Substrate Specificity
  • Transferases (Other Substituted Phosphate Groups) / chemistry*
  • Transferases (Other Substituted Phosphate Groups) / genetics*
  • Transferases (Other Substituted Phosphate Groups) / isolation & purification


  • CRLS1 protein, human
  • Cardiolipins
  • Cations, Divalent
  • Cytidine Diphosphate Diglycerides
  • Membrane Proteins
  • Transferases (Other Substituted Phosphate Groups)
  • cardiolipin synthetase