Javascript is not enabled on this browser. This site will not work properly without Javascript.
PhosphoSitePlus Homepage Cell Signaling Technology
PhosphoSitePlus
HomeAbout PhosphoSiteUsing PhosphoSiteCuration ProcessContact
NIH-logos NIGMS Logo NIAAA Logo NCI Logo NIH Logo
Protein Page:
UGT1A4 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
UGT1A4 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. Defects in UGT1A4 are the cause of Gilbert syndrome (GILBS). Gilbert syndrome occurs as a consequence of reduced bilirubin transferase activity and is often detected in young adults with vague nonspecific complaints. Defects in UGT1A4 are the cause of Crigler-Najjar syndrome type 1 (CN1). CN1 patients have severe hyperbilirubinemia and usually die of kernicterus (bilirubin accumulation in the basal ganglia and brainstem nuclei) within the first year of life. CN1 inheritance is autosomal recessive. Defects in UGT1A4 are the cause of Crigler-Najjar syndrome type 2 (CN2). CN2 patients have less severe hyperbilirubinemia and usually survive into adulthood without neurologic damage. Phenobarbital, which induces the partially deficient glucuronyl transferase, can diminish the jaundice. CN2 inheritance is autosomal dominant. Belongs to the UDP-glycosyltransferase family. 1 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Xenobiotic Metabolism - drug metabolism - cytochrome P450; Lipid Metabolism - androgen and estrogen; Membrane protein, integral; Carbohydrate Metabolism - pentose and glucuronate interconversions; Transferase; Xenobiotic Metabolism - drug metabolism - other enzymes; EC 2.4.1.17; Cofactor and Vitamin Metabolism - retinol; Endoplasmic reticulum; Xenobiotic Metabolism - metabolism by cytochrome P450; Carbohydrate Metabolism - ascorbate and aldarate; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Carbohydrate Metabolism - starch and sucrose
Chromosomal Location of Human Ortholog: 2q37
Cellular Component: endoplasmic reticulum membrane; endoplasmic reticulum; integral to membrane
Molecular Function: protein homodimerization activity; retinoic acid binding; enzyme binding; glucuronosyltransferase activity; protein heterodimerization activity
Biological Process: xenobiotic metabolic process
Reference #:  P22310 (UniProtKB)
Alt. Names/Synonyms: bilirubin UDP-glucuronosyltransferase isozyme 2; Bilirubin-specific UDPGT isozyme 2; GNT1; hUG-BR2; UD14; UDP glucuronosyltransferase 1 family, polypeptide A4; UDP glycosyltransferase 1 family, polypeptide A4; UDP-glucuronosyltransferase 1-4; UDP-glucuronosyltransferase 1-D; UDP-glucuronosyltransferase 1A4; UDPGT; UDPGT 1-4; UGT-1D; UGT1; UGT1*4; UGT1-04; UGT1.4; UGT1A4; UGT1D
Gene Symbols: UGT1A4
Molecular weight: 60,025 Da
Basal Isoelectric point: 8.79  Predict pI for various phosphorylation states
Select Structure to View Below

UGT1A4

Protein Structure Not Found.


STRING  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  DISEASE  |  Scansite  |  Pfam  |  ENZYME  |  Phospho.ELM  |  Source  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene


Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 T14-p VPLPRLAtGLLLLLs
0 1 S21-p tGLLLLLsVQPWAES
0 1 K70 PEVNMHIKEEKFFTL
0 1 T78 EEKFFTLTAYAVPWT
0 1 K108 FETEHLLKRYSRSMA
0 1 R112 HLLKRYSRSMAIMNN
0 1 R112 HLLKRYSRSMAIMNN
0 11 K354 ANNTILVKWLPQNDL
0 1 K438-ac VINDKSYkENIMRLS
  mouse

 
G12 VPLQGLVGLLLLCAL
C17 LVGLLLLCALPWTEG
K66-ub PEVNMRIkEEDFFTF
K74-ac EEDFFTFkVYAVPYT
K103-sc FDTGNYMkKIFkTSE
K107-ac NYMkKIFkTSEALRN
K107-sc NYMkKIFkTSEALRN
K349-ac AKNTILVkWLPQNDL
K433 VINNKSYKENIMRLS
Home  |  Curator Login With enhanced literature mining using Linguamatics I2E I2E Logo Produced by 3rd Millennium  |  Design by Digizyme
©2003-2013 Cell Signaling Technology, Inc.