CDP-diacylglycerol—glycerol-3-phosphate 3-phosphatidyltransferase

CDP-diacylglycerol—glycerol-3-phosphate 3-phosphatidyltransferase is an enzyme that catalyzes the chemical reaction in the phospholipid biosynthetic pathway. This enzyme belongs to the family of transferase enzymes, specifically those transferring non-standard substituted phosphate groups.

Function

CDP-diacylglycerol—glycerol-3-phosphate 3-phosphatidyltransferase, also known as phosphatidate cytidylyltransferase, is involved in the synthesis of phosphatidylglycerol (PG), a critical component of the inner mitochondrial membrane. The enzyme catalyzes the transfer of the phosphatidyl group of CDP-diacylglycerol to glycerol-3-phosphate, forming PG and CMP.

Structure

The enzyme is a membrane protein and is located in the mitochondria of cells. The exact structure of the enzyme is not yet fully understood, but it is known to contain multiple transmembrane domains.

Clinical significance

Defects in this enzyme can lead to a variety of diseases, including Barth syndrome, a rare genetic disorder characterized by cardiomyopathy, neutropenia, and growth delay.

See also

• Phosphatidylglycerol
• Phospholipid
• Transferase