- Human pathology

Home > A. Molecular pathology > mitochondrial phospholipids

mitochondrial phospholipids

Friday 22 October 2004

Mitochondrial phospholipids comprise a significant proportion of the entire phospholipid content of most eukaroytic cells.

In the heart, a tissue rich in mitochondria, the mitochondrial phospholipids provide for diverse roles in the regulation of various mitochondrial processes including apoptosis, electron transport, and mitochondrial lipid and protein import.


- alteration in the content and fatty acid composition of phospholipids within the heart is linked to alterations in myocardial electrical activity.
- reduction in the specific mitochondrial phospholipid cardiolipin is an underlying biochemical cause of Barth syndrome, a rare and often fatal X-linked genetic disease that is associated with cardiomyopathy by mutations in the tafazzin (TAZ) gene.


- Hatch GM. Cell biology of cardiac mitochondrial phospholipids. Biochem Cell Biol. 2004 Feb;82(1):99-112. PMID: 15052331