UniProtKB - Q9P2W7 (B3GA1_HUMAN)
Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 1
B3GAT1
Functioni
Involved in the biosynthesis of L2/HNK-1 carbohydrate epitope on glycoproteins. Can also play a role in glycosaminoglycan biosynthesis. Substrates include asialo-orosomucoid (ASOR), asialo-fetuin, and asialo-neural cell adhesion molecule. Requires sphingomyelin for activity: stearoyl-sphingomyelin was the most effective, followed by palmitoyl-sphingomyelin and lignoceroyl-sphingomyelin. Activity was demonstrated only for sphingomyelin with a saturated fatty acid and not for that with an unsaturated fatty acid, regardless of the length of the acyl group.
By similarityCatalytic activityi
- 3-O-(β-D-galactosyl-(1→3)-β-D-galactosyl-(1→4)-β-D-xylosyl)-L-seryl-[protein] + UDP-α-D-glucuronate = 3-O-(β-D-GlcA-(1→3)-β-D-Gal-(1→3)-β-D-Gal-(1→4)-β-D-Xyl)-L-seryl-[protein] + H+ + UDPBy similarityEC:2.4.1.135By similarity
Cofactori
: protein glycosylation Pathwayi
This protein is involved in the pathway protein glycosylation, which is part of Protein modification.View all proteins of this organism that are known to be involved in the pathway protein glycosylation and in Protein modification.
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 122 | UDP-glucuronate | 1 | |
Binding sitei | 165 | UDP-glucuronate | 1 | |
Binding sitei | 170 | UDP-glucuronateBy similarity | 1 | |
Metal bindingi | 197 | ManganeseBy similarity | 1 | |
Active sitei | 284 | Proton donor/acceptor | 1 |
Regions
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Nucleotide bindingi | 91 – 93 | UDP-glucuronate binding | 3 | |
Nucleotide bindingi | 195 – 197 | UDP-glucuronate binding | 3 | |
Nucleotide bindingi | 311 – 313 | UDP-glucuronate binding | 3 |
GO - Molecular functioni
- galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity Source: UniProtKB
- metal ion binding Source: UniProtKB-KW
- UDP-galactose:beta-N-acetylglucosamine beta-1,3-galactosyltransferase activity Source: Ensembl
GO - Biological processi
- carbohydrate metabolic process Source: GO_Central
- chondroitin sulfate proteoglycan biosynthetic process Source: GO_Central
- protein glycosylation Source: UniProtKB-UniPathway
Keywordsi
Molecular function | Transferase |
Ligand | Manganese, Metal-binding |
Enzyme and pathway databases
BRENDAi | 2.4.1.135, 2681 |
PathwayCommonsi | Q9P2W7 |
Reactomei | R-HSA-1971475, A tetrasaccharide linker sequence is required for GAG synthesis |
SignaLinki | Q9P2W7 |
UniPathwayi | UPA00378 |
Protein family/group databases
CAZyi | GT43, Glycosyltransferase Family 43 |
Names & Taxonomyi
Protein namesi | Recommended name: Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 1Curated (EC:2.4.1.135By similarity)Alternative name(s): Beta-1,3-glucuronyltransferase 1 Glucuronosyltransferase P Short name: GlcAT-PBy similarity UDP-GlcUA:glycoprotein beta-1,3-glucuronyltransferase Short name: GlcUAT-P |
Gene namesi | |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:921, B3GAT1 |
MIMi | 151290, gene |
neXtProti | NX_Q9P2W7 |
VEuPathDBi | HostDB:ENSG00000109956 |
Subcellular locationi
Extracellular region or secreted
- Secreted By similarity
Golgi apparatus
- Golgi apparatus membrane By similarity; Single-pass type II membrane protein By similarity
Extracellular region or secreted
- Secreted By similarity
Golgi apparatus
- Golgi apparatus membrane By similarity; Single-pass type II membrane protein By similarity
Endoplasmic reticulum
- Endoplasmic reticulum membrane By similarity
Endoplasmic reticulum
- endoplasmic reticulum membrane Source: UniProtKB
Extracellular region or secreted
- extracellular region Source: UniProtKB-SubCell
Golgi apparatus
- Golgi membrane Source: UniProtKB
Other locations
- integral component of membrane Source: ProtInc
- intracellular membrane-bounded organelle Source: HPA
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Topological domaini | 1 – 6 | CytoplasmicSequence analysis | 6 | |
Transmembranei | 7 – 27 | Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST | 21 | |
Topological domaini | 28 – 334 | LumenalSequence analysisAdd BLAST | 307 |
Keywords - Cellular componenti
Endoplasmic reticulum, Golgi apparatus, Membrane, SecretedPathology & Biotechi
Organism-specific databases
DisGeNETi | 27087 |
OpenTargetsi | ENSG00000109956 |
PharmGKBi | PA25215 |
Miscellaneous databases
Pharosi | Q9P2W7, Tbio |
Chemistry databases
DrugBanki | DB01694, D-tartaric acid DB03435, Uridine-5'-Diphosphate |
Genetic variation databases
BioMutai | B3GAT1 |
DMDMi | 205830910 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000195167 | 1 – 334 | Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 1Add BLAST | 334 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 103 | PhosphothreonineBy similarity | 1 | |
Modified residuei | 108 | PhosphothreonineBy similarity | 1 | |
Glycosylationi | 140 | N-linked (GlcNAc...) asparagineSequence analysis | 1 | |
Glycosylationi | 184 | N-linked (GlcNAc...) asparagineSequence analysis | 1 | |
Glycosylationi | 303 | N-linked (GlcNAc...) asparagineSequence analysis | 1 |
Post-translational modificationi
Keywords - PTMi
Glycoprotein, PhosphoproteinProteomic databases
jPOSTi | Q9P2W7 |
MassIVEi | Q9P2W7 |
PaxDbi | Q9P2W7 |
PeptideAtlasi | Q9P2W7 |
PRIDEi | Q9P2W7 |
ProteomicsDBi | 83905 |
PTM databases
GlyConnecti | 1254, 1 N-Linked glycan (1 site) |
GlyGeni | Q9P2W7, 3 sites, 1 N-linked glycan (1 site) |
iPTMneti | Q9P2W7 |
PhosphoSitePlusi | Q9P2W7 |
Expressioni
Tissue specificityi
Gene expression databases
Bgeei | ENSG00000109956, Expressed in cortical plate and 149 other tissues |
Genevisiblei | Q9P2W7, HS |
Organism-specific databases
HPAi | ENSG00000109956, Tissue enriched (brain) |
Interactioni
Subunit structurei
Homodimer.
Interacts with SAR1A.
By similaritySites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sitei | 228 | Interaction with galactose moiety of substrate glycoprotein | 1 | |
Sitei | 321 | Interaction with galactose moiety of substrate glycoproteinBy similarity | 1 |
Binary interactionsi
Q9P2W7
With | #Exp. | IntAct |
---|---|---|
CMTM7 [Q96FZ5] | 3 | EBI-3918235,EBI-2807956 |
Protein-protein interaction databases
BioGRIDi | 117990, 391 interactors |
IntActi | Q9P2W7, 3 interactors |
STRINGi | 9606.ENSP00000433847 |
Miscellaneous databases
RNActi | Q9P2W7, protein |
Structurei
Secondary structure
3D structure databases
AlphaFoldDBi | Q9P2W7 |
SMRi | Q9P2W7 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | Q9P2W7 |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 3 – 5 | Essential for transport from endoplasmic reticulum to Golgi apparatus and interaction with SAR1ABy similarity | 3 | |
Regioni | 37 – 56 | DisorderedSequence analysisAdd BLAST | 20 | |
Regioni | 245 – 254 | Interaction with galactose moiety of substrate glycoprotein | 10 |
Sequence similaritiesi
Keywords - Domaini
Signal-anchor, Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG1476, Eukaryota |
GeneTreei | ENSGT00940000157165 |
HOGENOMi | CLU_045177_1_0_1 |
InParanoidi | Q9P2W7 |
OMAi | DSREYCM |
OrthoDBi | 901158at2759 |
PhylomeDBi | Q9P2W7 |
TreeFami | TF313522 |
Family and domain databases
CDDi | cd00218, GlcAT-I, 1 hit |
Gene3Di | 3.90.550.10, 1 hit |
InterProi | View protein in InterPro IPR005027, Glyco_trans_43 IPR029044, Nucleotide-diphossugar_trans |
PANTHERi | PTHR10896, PTHR10896, 1 hit |
Pfami | View protein in Pfam PF03360, Glyco_transf_43, 1 hit |
SUPFAMi | SSF53448, SSF53448, 1 hit |
s (2)i Sequence
Sequence statusi: Complete.
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MPKRRDILAI VLIVLPWTLL ITVWHQSTLA PLLAVHKDEG SDPRRETPPG
60 70 80 90 100
ADPREYCTSD RDIVEVVRTE YVYTRPPPWS DTLPTIHVVT PTYSRPVQKA
110 120 130 140 150
ELTRMANTLL HVPNLHWLVV EDAPRRTPLT ARLLRDTGLN YTHLHVETPR
160 170 180 190 200
NYKLRGDARD PRIPRGTMQR NLALRWLRET FPRNSSQPGV VYFADDDNTY
210 220 230 240 250
SLELFEEMRS TRRVSVWPVA FVGGLRYEAP RVNGAGKVVG WKTVFDPHRP
260 270 280 290 300
FAIDMAGFAV NLRLILQRSQ AYFKLRGVKG GYQESSLLRE LVTLNDLEPK
310 320 330
AANCTKILVW HTRTEKPVLV NEGKKGFTDP SVEI
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 240 | G → R in BAA96077 (PubMed:10783264).Curated | 1 | |
Sequence conflicti | 267 | Q → K in BAH13084 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 306 | K → R in BAH13084 (PubMed:14702039).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_044538 | 131 | A → T. Corresponds to variant dbSNP:rs35434644Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_058538 | 1 | M → MGNEEPWVQPALEM in isoform 2. | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AB029396 mRNA Translation: BAA96077.1 AK299637 mRNA Translation: BAH13084.1 CR457098 mRNA Translation: CAG33379.1 BC010466 mRNA Translation: AAH10466.1 |
CCDSi | CCDS8500.1 [Q9P2W7-1] |
RefSeqi | NP_061114.2, NM_018644.3 [Q9P2W7-1] NP_473366.1, NM_054025.2 [Q9P2W7-1] XP_005271563.1, XM_005271506.3 XP_011541053.1, XM_011542751.2 [Q9P2W7-2] XP_011541055.1, XM_011542753.2 [Q9P2W7-2] XP_016873039.1, XM_017017550.1 [Q9P2W7-1] XP_016873040.1, XM_017017551.1 [Q9P2W7-2] |
Genome annotation databases
Ensembli | ENST00000312527.9; ENSP00000307875.4; ENSG00000109956.13 ENST00000392580.5; ENSP00000376359.1; ENSG00000109956.13 ENST00000524765.1; ENSP00000433847.1; ENSG00000109956.13 |
GeneIDi | 27087 |
KEGGi | hsa:27087 |
MANE-Selecti | ENST00000312527.9; ENSP00000307875.4; NM_054025.3; NP_473366.1 |
UCSCi | uc001qhq.4, human [Q9P2W7-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AB029396 mRNA Translation: BAA96077.1 AK299637 mRNA Translation: BAH13084.1 CR457098 mRNA Translation: CAG33379.1 BC010466 mRNA Translation: AAH10466.1 |
CCDSi | CCDS8500.1 [Q9P2W7-1] |
RefSeqi | NP_061114.2, NM_018644.3 [Q9P2W7-1] NP_473366.1, NM_054025.2 [Q9P2W7-1] XP_005271563.1, XM_005271506.3 XP_011541053.1, XM_011542751.2 [Q9P2W7-2] XP_011541055.1, XM_011542753.2 [Q9P2W7-2] XP_016873039.1, XM_017017550.1 [Q9P2W7-1] XP_016873040.1, XM_017017551.1 [Q9P2W7-2] |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
1V82 | X-ray | 1.85 | A/B | 83-334 | [»] | |
1V83 | X-ray | 1.90 | A/B | 83-334 | [»] | |
1V84 | X-ray | 1.82 | A/B | 83-334 | [»] | |
AlphaFoldDBi | Q9P2W7 | |||||
SMRi | Q9P2W7 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 117990, 391 interactors |
IntActi | Q9P2W7, 3 interactors |
STRINGi | 9606.ENSP00000433847 |
Chemistry databases
DrugBanki | DB01694, D-tartaric acid DB03435, Uridine-5'-Diphosphate |
Protein family/group databases
CAZyi | GT43, Glycosyltransferase Family 43 |
PTM databases
GlyConnecti | 1254, 1 N-Linked glycan (1 site) |
GlyGeni | Q9P2W7, 3 sites, 1 N-linked glycan (1 site) |
iPTMneti | Q9P2W7 |
PhosphoSitePlusi | Q9P2W7 |
Genetic variation databases
BioMutai | B3GAT1 |
DMDMi | 205830910 |
Proteomic databases
jPOSTi | Q9P2W7 |
MassIVEi | Q9P2W7 |
PaxDbi | Q9P2W7 |
PeptideAtlasi | Q9P2W7 |
PRIDEi | Q9P2W7 |
ProteomicsDBi | 83905 |
Protocols and materials databases
Antibodypediai | 3739, 1557 antibodies from 45 providers |
DNASUi | 27087 |
Genome annotation databases
Ensembli | ENST00000312527.9; ENSP00000307875.4; ENSG00000109956.13 ENST00000392580.5; ENSP00000376359.1; ENSG00000109956.13 ENST00000524765.1; ENSP00000433847.1; ENSG00000109956.13 |
GeneIDi | 27087 |
KEGGi | hsa:27087 |
MANE-Selecti | ENST00000312527.9; ENSP00000307875.4; NM_054025.3; NP_473366.1 |
UCSCi | uc001qhq.4, human [Q9P2W7-1] |
Organism-specific databases
CTDi | 27087 |
DisGeNETi | 27087 |
GeneCardsi | B3GAT1 |
HGNCi | HGNC:921, B3GAT1 |
HPAi | ENSG00000109956, Tissue enriched (brain) |
MIMi | 151290, gene |
neXtProti | NX_Q9P2W7 |
OpenTargetsi | ENSG00000109956 |
PharmGKBi | PA25215 |
VEuPathDBi | HostDB:ENSG00000109956 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG1476, Eukaryota |
GeneTreei | ENSGT00940000157165 |
HOGENOMi | CLU_045177_1_0_1 |
InParanoidi | Q9P2W7 |
OMAi | DSREYCM |
OrthoDBi | 901158at2759 |
PhylomeDBi | Q9P2W7 |
TreeFami | TF313522 |
Enzyme and pathway databases
UniPathwayi | UPA00378 |
BRENDAi | 2.4.1.135, 2681 |
PathwayCommonsi | Q9P2W7 |
Reactomei | R-HSA-1971475, A tetrasaccharide linker sequence is required for GAG synthesis |
SignaLinki | Q9P2W7 |
Miscellaneous databases
BioGRID-ORCSi | 27087, 9 hits in 1068 CRISPR screens |
ChiTaRSi | B3GAT1, human |
EvolutionaryTracei | Q9P2W7 |
GeneWikii | B3GAT1 |
GenomeRNAii | 27087 |
Pharosi | Q9P2W7, Tbio |
PROi | PR:Q9P2W7 |
RNActi | Q9P2W7, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000109956, Expressed in cortical plate and 149 other tissues |
Genevisiblei | Q9P2W7, HS |
Family and domain databases
CDDi | cd00218, GlcAT-I, 1 hit |
Gene3Di | 3.90.550.10, 1 hit |
InterProi | View protein in InterPro IPR005027, Glyco_trans_43 IPR029044, Nucleotide-diphossugar_trans |
PANTHERi | PTHR10896, PTHR10896, 1 hit |
Pfami | View protein in Pfam PF03360, Glyco_transf_43, 1 hit |
SUPFAMi | SSF53448, SSF53448, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | B3GA1_HUMAN | |
Accessioni | Q9P2W7Primary (citable) accession number: Q9P2W7 Secondary accession number(s): B7Z5Z8, Q96FS7 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | June 1, 2001 |
Last sequence update: | July 22, 2008 | |
Last modified: | May 25, 2022 | |
This is version 194 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. |
Miscellaneousi
Keywords - Technical termi
3D-structure, Reference proteomeDocuments
- Human chromosome 11
Human chromosome 11: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families