NP_000454.1
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
49,368 Da
NCBI Official Full Name
UDP-glucuronosyltransferase 1-1
NCBI Official Synonym Full Names
UDP glucuronosyltransferase 1 family, polypeptide A1
NCBI Official Synonym Symbols
GNT1; UGT1; UDPGT; UGT1A; HUG-BR1; BILIQTL1; UDPGT 1-1 [Similar Products]
NCBI Protein Information
UDP-glucuronosyltransferase 1-1
UniProt Protein Name
UDP-glucuronosyltransferase 1-1
UniProt Synonym Protein Names
Bilirubin-specific UDPGT isozyme 1; hUG-BR1; UDP-glucuronosyltransferase 1-A; UGT-1A; UGT1A; UDP-glucuronosyltransferase 1A1
UniProt Synonym Gene Names
GNT1; UGT1; UDPGT 1-1; UGT1*1; UGT1-01; UGT1.1; hUG-BR1; UGT-1A; UGT1A [Similar Products]
UniProt Entry Name
UD11_HUMAN
NCBI Summary for UGT1A1
This gene encodes a UDP-glucuronosyltransferase, an enzyme of the glucuronidation pathway that transforms small lipophilic molecules, such as steroids, bilirubin, hormones, and drugs, into water-soluble, excretable metabolites. This gene is part of a complex locus that encodes several UDP-glucuronosyltransferases. The locus includes thirteen unique alternate first exons followed by four common exons. Four of the alternate first exons are considered pseudogenes. Each of the remaining nine 5' exons may be spliced to the four common exons, resulting in nine proteins with different N-termini and identical C-termini. Each first exon encodes the substrate binding site, and is regulated by its own promoter. The preferred substrate of this enzyme is bilirubin, although it also has moderate activity with simple phenols, flavones, and C18 steroids. Mutations in this gene result in Crigler-Najjar syndromes types I and II and in Gilbert syndrome. [provided by RefSeq, Jul 2008]
UniProt Comments for UGT1A1
UGT1A1: UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform glucuronidates bilirubin IX- alpha to form both the IX-alpha-C8 and IX-alpha-C12 monoconjugates and diconjugate. Is also able to catalyze the glucuronidation of 17beta-estradiol, 17alpha-ethinylestradiol, 1-hydroxypyrene, 4- methylumbelliferone, 1-naphthol, paranitrophenol, scopoletin, and umbelliferone. Part a large chaperone multiprotein complex comprising DNAJB11, HSP90B1, HSPA5, HYOU, PDIA2, PDIA4, PDIA6, PPIB, SDF2L1, UGT1A1 and very small amounts of ERP29, but not, or at very low levels, CALR nor CANX. Expressed in liver. Not expressed in skin or kidney. Belongs to the UDP-glycosyltransferase family. 1 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 2.4.1.17; Membrane protein, integral; Carbohydrate Metabolism - starch and sucrose; Transferase; Lipid Metabolism - androgen and estrogen; Xenobiotic Metabolism - drug metabolism - other enzymes; Xenobiotic Metabolism - drug metabolism - cytochrome P450; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Carbohydrate Metabolism - ascorbate and aldarate; Xenobiotic Metabolism - metabolism by cytochrome P450; Cofactor and Vitamin Metabolism - retinol; Carbohydrate Metabolism - pentose and glucuronate interconversions
Chromosomal Location of Human Ortholog: 2q37
Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; integral to plasma membrane
Molecular Function: enzyme binding; enzyme inhibitor activity; glucuronosyltransferase activity; protein heterodimerization activity; protein homodimerization activity; retinoic acid binding; steroid binding
Biological Process: acute-phase response; bilirubin conjugation; digestion; drug metabolic process; estrogen metabolic process; flavone metabolic process; flavonoid biosynthetic process; heme catabolic process; heterocycle metabolic process; liver development; negative regulation of catalytic activity; negative regulation of fatty acid metabolic process; negative regulation of steroid metabolic process; organ regeneration; porphyrin metabolic process; response to drug; response to lipopolysaccharide; response to nutrient; response to starvation; retinoic acid metabolic process; steroid metabolic process; xenobiotic metabolic process
Disease: Bilirubin, Serum Level Of, Quantitative Trait Locus 1; Crigler-najjar Syndrome, Type I; Crigler-najjar Syndrome, Type Ii; Gilbert Syndrome; Hyperbilirubinemia, Transient Familial Neonatal
Research Articles on UGT1A1
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Organs/Tissues associated with UGT1A1 elisa kit
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