Fig. 1: After phosphatidic acid (PA) is imported into the matrix, the cardiolipin biosynthesis process begins. | Cell Death & Disease

Fig. 1: After phosphatidic acid (PA) is imported into the matrix, the cardiolipin biosynthesis process begins.

From: The role of cardiolipin in mitochondrial dynamics and quality control in neuronal ischemia/reperfusion injury

Fig. 1

Biosynthesis begins with PA being converted to cytidine diphosphate diacylglycerol (CDP-DAG) by the membrane bound protein Tamm41. CDP-DAG then interacts with phosphatidyl glycerophosphate synthase 1 (PGS1), another membrane bound protein, producing p-glycoprotein (PGP). Protein tyrosine phosphatase mitochondrial 1 (PTPMT1) then converts PGP to PG. Biosynthesis is then concluded with cardiolipin synthase (CRLS1) converts PG to premature cardiolipin (CL). Once premature cardiolipin is produced the remodeling process begins. A membrane of the iPLA2 family cleaves one of the fatty acyl chains of the premature cardiolipin and replaces it with a hydroxyl group producing the intermediate monolysocardiolipin (MLCL). Tafazzin then re-acylates monolysocardiolipin with an unsaturated fatty acid chain producing the more stable mature CL. The main difference between premature and mature cardiolipin is the degree of saturation, with the mature form consisting of more unsaturated fatty acid tails resulting in a more stable cardiolipin.

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