UDP glucuronosyl transferase
UDP-GT1-A1 | ||
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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 .