Cellular and Molecular Life Sciences

, Volume 65, Issue 16, pp 2493–2506

Cardiolipin, the heart of mitochondrial metabolism


DOI: 10.1007/s00018-008-8030-5

Cite this article as:
Houtkooper, R.H. & Vaz, F.M. Cell. Mol. Life Sci. (2008) 65: 2493. doi:10.1007/s00018-008-8030-5


Cardiolipin is a unique phospholipid, which is almost exclusively localized in the mitochondrial inner membrane where it is synthesized from phosphatidylglycerol and cytidinediphosphate-diacylglycerol. After primary synthesis, the mature acyl chain composition of cardiolipin is achieved by at least two remodeling mechanisms. In the mitochondrial membrane cardiolipin plays an important role in energy metabolism, mainly by providing stability for the individual enzymes and enzyme complexes involved in energy production. Moreover, cardiolipin is involved in different stages of the mitochondrial apoptotic process and in mitochondrial membrane dynamics. Cardiolipin alterations have been described in various pathological conditions. Patients suffering from Barth syndrome have an altered cardiolipin homeostasis caused by a primary deficiency in cardiolipin remodeling. Alterations in cardiolipin content or composition have also been reported in more frequent diseases such as diabetes and heart failure. In this review we provide an overview of cardiolipin metabolism, function and its role in different pathological states.


Cardiolipinphospholipidacyl chain compositionenergy productionmitochondrial apoptosisBarth syndrome

Copyright information

© Birkhaueser 2008

Authors and Affiliations

  1. 1.Academic Medical Center, Departments of Clinical Chemistry and PediatricsLaboratory of Genetic Metabolic Diseases, University of AmsterdamAmsterdamThe Netherlands