Metabolism and function of mitochondrial cardiolipin

Prog Lipid Res. 2014 Jul:55:1-16. doi: 10.1016/j.plipres.2014.04.001. Epub 2014 Apr 24.


Since it has been recognized that mitochondria are crucial not only for energy metabolism but also for other cellular functions, there has been a growing interest in cardiolipin, the specific phospholipid of mitochondrial membranes. Indeed, cardiolipin is a universal component of mitochondria in all eukaryotes. It has a unique dimeric structure comprised of two phosphatidic acid residues linked by a glycerol bridge, which gives rise to unique physicochemical properties. Cardiolipin plays an important role in the structural organization and the function of mitochondrial membranes. In this article, we review the literature on cardiolipin biology, focusing on the most important discoveries of the past decade. Specifically, we describe the formation, the migration, and the degradation of cardiolipin and we discuss how cardiolipin affects mitochondrial function. We also give an overview of the various phenotypes of cardiolipin deficiency in different organisms.

Keywords: Cardiolipin; Disease; Fatty acids; Membranes; Mitochondria; Phospholipids.

Publication types

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

MeSH terms

  • Animals
  • Apoptosis
  • Biological Transport
  • Cardiolipins / metabolism*
  • Humans
  • Mitochondria / metabolism*
  • Mitochondrial Membranes / metabolism
  • Signal Transduction


  • Cardiolipins