Figure 1: Cardiolipin synthesis and remodeling pathway in humans and yeast. Phosphatidic acid (PA) is converted to CDP-diacylglycerol (CDP-DAG) by CDP-DAG synthase. Phosphatidylglycerolphosphate synthase catalyzes the conversion of CDP-DAG to phosphatidylglycerolphosphate (PGP), which is dephosphorylated to phosphatidylglycerol (PG). PG is converted to unremodeled CL with mostly saturated acyl chains (). is deacylated to monolyso-CL (MLCL) by phospholipases and MLCL is reacylated to CL with mostly unsaturated acyl chains (). The genes encoding human enzymes are indicated in red, and genes that encode yeast enzymes are in blue.