Cardiolipin, an important phospholipid in the mitochondrial membranes, is synthesized via condensation of phosphatidylglycerol (PG) and CDP-diacylglycerol catalysed by cardiolipin synthase (CLS) in the matrix side of the inner mitochondrial membrane (Figure 2).
Studies have indicated that both lysophosphatidic acid and phosphatidic acid are synthesized on the outer surface of the mitochondrial outer membrane and then phosphatidic acid moves to the inner mitochondrial membrane where it serves as a precursor for cardiolipin biosynthesis .
Remodeling of cardiolipin has been extensively studied due to its role in the functionality of the mitochondria.
Another enzyme involved in cardiolipin remodeling is acyl coenzyme A thioesterase (Them5).
Because, its role in cardiolipin remodeling, the hippocampus of iPLA2[gamma] null mice shows an elevated content of mitochondrial cardiolipin with an altered chain length composition .
Since cytochrome c is attached to cardiolipin in the mitochondrial intermembrane space , cardiolipin deficiency would be expected to cause the release of cytochrome c into the cytoplasm.
Tafazzin plays an important role in cardiolipin remodeling in the mitochondria.
Cardiolipin is essential for proper function of the mitochondrial respiration chain, and dysfunction of the chain complexes leads to increased production of ROS .
Though cardiolipin deficiency directly results in the dissociation of cytochrome c from cardiolipin into the intermembrane space of mitochondria, extrusion of this protein still needs permeabilization of the outer mitochondrial membrane .
Schlame, "Cardiolipin remodeling and the function of tafazzin," Biochimica etBiophysica Acta, vol.
Cardiolipin deficiency in X-linked cardioskeletal myopathy and neutropenia (Barth syndrome, MIM 302060): a study in cultured skin fibroblasts.
 Nonstandard abbreviations: BTHS, Barth syndrome; CL, cardiolipin; MLCL, monolysocardiolipin; PC, phosphatidylcholine; MS/MS, tandem mass spectrometry; MRM, multiple reaction monitoring; S/N, signal-to-noise ratio.