Skip to main content
. 2022 Apr 8;13(4):656. doi: 10.3390/genes13040656

Figure 2.

Figure 2

Cardiolipin (CL) de novo synthesis and remodeling pathways. Glyceraldehyde 3-phosphate (G-3-P) is catalyzed by a series of enzymes to generate nascent CL. To achieve a final symmetric acyl composition (e.g., TetralinoleoyI-CL in the heart), nascent CL undergoes an extensive remodeling process by deacylation to monolyso-CL (MLCL), and subsequent reacylation catalyzed by specific acyltransferases including Tafazzin (TAZ). GPAT: glycerol-3-phosphate acyltransferase; AGPAT: 1-acylglycerol-3-phosphate-O-acyltransferase; PA: phosphatidic acid; CDS: cytidine diphosphate diacylglycerol synthases; CDP-DAG: cytidine diphosphate-diacylglycerol; PGP: phosphatidylglycerol phosphate; PG: phosphatidylglycerol; PGPS: PGP synthase; PTPMT1: protein tyrosine phosphatase mitochondrial 1; CLS: CL synthase; PLA2: phospholipase A2; MLCL: monolyso-CL; PC: phosphatidylcholine; LPC: lysophosphocholine; ALCAT1: acyl-CoA:lysocardiolipin acyltransferase 1; MLCLAT1: monolysocardiolipin acyltransferase 1. Light blue box: glycerol backbone; Red-down arrows (↓): decreased in BTHS; Red-up arrow (↑): increased in BTHS; Green content: Linoleoyl groups; Red cross (X): TAZ loss of function in BTHS.