The role of mitochondrial cardiolipin in heart function and its implication in cardiac disease

https://doi.org/10.1016/j.bbadis.2018.08.025Get rights and content
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Highlights

  • Tafazzin mediates remodeling of the phospholipid Cardiolipin in mitochondrial membranes.

  • Cardiolipin is required for mitochondrial respiration, providing 95% of the cardiac energy demand.

  • Other functions of Cardiolipin include morphology, mitophagy and apoptosis.

  • Changes in Cardiolipin pool are associated with cardiomyopathy in Barth syndrome (BTHS), and Sengers disease.

Abstract

Mitochondria play an essential role in the energy metabolism of the heart. Many of the essential functions are associated with mitochondrial membranes and oxidative phosphorylation driven by the respiratory chain. Mitochondrial membranes are unique in the cell as they contain the phospholipid cardiolipin. The important role of cardiolipin in cardiovascular health is highlighted by several cardiac diseases, in which cardiolipin plays a fundamental role. Barth syndrome, Sengers syndrome, and Dilated cardiomyopathy with ataxia (DCMA) are genetic disorders, which affect cardiolipin biosynthesis. Other cardiovascular diseases including ischemia/reperfusion injury and heart failure are also associated with changes in the cardiolipin pool. Here, we summarize molecular functions of cardiolipin in mitochondrial biogenesis and morphology. We highlight the role of cardiolipin for the respiratory chain, metabolite carriers, and mitochondrial metabolism and describe links to apoptosis and mitochondria specific autophagy (mitophagy) with possible implications in cardiac disease.

Abbreviations

MAM
mitochondrial associated membrane
OM
outer membrane
IM
inner membrane
IMS
intermembrane space
CL
cardiolipin
PE
phosphatidylethanolamine
PS
phosphatidylserine
PA
phosphatidic acid

Keywords

Mitochondria
Cardiolipin
Respiratory chain
Barth syndrome
Sengers syndrome

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This article is part of a Special Issue entitled: The power of metabolism: Linking energy supply and demand to contractile function edited by Torsten Doenst, Michael Schwarzer and Christine Des Rosiers.