UDP glucuronosyl transferase
| UDP-GT1-A1 | ||
|---|---|---|
| Properties of human protein | ||
| Mass / length primary structure | 508 amino acids | |
| Secondary to quaternary structure | single pass membrane protein | |
| Identifier | ||
| Gene name | UGT1A1 | |
| External IDs | ||
| Enzyme classification | ||
| EC, category | 2.4.1.17 , glycosyl transferase | |
| Response type | Glycosylation | |
| Substrate | Bilirubin + UDP-glucuronate | |
| Products | Bilirubin monoglucuronoside + UDP | |
| Occurrence | ||
| Homology family | Glycosyl transferase | |
| Parent taxon | Chordates | |
UDP-glucuronosyltransferase or UDP-glucuronyltransferase (UDP-GT) is the name for enzymes that transfer glucuronic acid from UDP-glucuronate to a small, hydrophobic molecule ( glucuronidation ). They are important in breaking down harmful substances in the body. In particular, the human enzyme UDP-GT1-A1 is required in large quantities in the liver for porphyrin degradation. A lack of functional UDP-GT1-A1 leads to hyperbilirubinemia and thus to the (relatively harmless) disease Meulengracht's disease . Rare mutations in the UGT1A1 - gene , the stark to (almost) defect cause of the enzyme that are very hard running form of the disease, the cause Crigler-Najjar syndrome .
The UDP-GT belong to the UDP-glycosyltransferases (UGT), enzymes that transfer a glycosyl (sugar) group from one UTP molecule to another small hydrophobic molecule. These enzymes are found in viruses , bacteria , plants and animals , where they are an important part of the metabolism .
