The topology and regulation of cardiolipin biosynthesis and remodeling in yeast

Chem Phys Lipids. 2014 Apr;179:25-31. doi: 10.1016/j.chemphyslip.2013.10.008. Epub 2013 Nov 1.


The signature mitochondrial phospholipid cardiolipin plays an important role in mitochondrial function, and alterations in cardiolipin metabolism are associated with human disease. Topologically, cardiolipin biosynthesis and remodeling are complex. Precursor phospholipids must be transported from the ER, across the mitochondrial outer membrane to the matrix-facing leaflet of the inner membrane, where cardiolipin biosynthesis commences. Post-synthesis, cardiolipin undergoes acyl chain remodeling, requiring additional trafficking steps, before it achieves its final distribution within both mitochondrial membranes. This process is regulated at several points via multiple independent mechanisms. Here, we review the regulation and topology of cardiolipin biosynthesis and remodeling in the yeast Saccharomyces cerevisiae. Although cardiolipin metabolism is more complicated in mammals, yeast have been an invaluable model for dissecting the steps required for this process.

Keywords: Cardiolipin; Lipid trafficking; Mitochondria; Regulation; Remodeling; Yeast.

Publication types

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

MeSH terms

  • Cardiolipins / biosynthesis*
  • Cardiolipins / metabolism*
  • Saccharomyces cerevisiae / metabolism*


  • Cardiolipins