Figure 5.
Cardiolipin remodeling by lysocardiolipin acyltransferase in pulmonary fibrosis. The mitochondrial phospholipid cardiolipin is converted by iPLA2 or cPLA2 to monolysocardiolipin (MLCL), a regulator of apoptosis, in the mitochondria. Dysregulation of LYCAT function, which remodels the fatty acid composition of cardiolipin, has been implicated in pulmonary fibrosis and Barth syndrome. ALCAT1—Acyl-CoA: lysocardiolipin acyltransferase, CoA—CoenzymeA, FA—Fatty acid, LYCAT—lysocardiolipin acyltransferase, LPC—lysophosphatidylcholine, LPE—lysophosphatidylethanolamine, PC—Phosphatidylcholine, PE—Phosphatidylethanolamine, iPLA2—calcium-independent Phospholipase A2, cPLA2—cytosolic phospholipase A2.