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UniProtKB - U3KPV4 (A3LT2_HUMAN)
Protein
Alpha-1,3-galactosyltransferase 2
Gene
A3GALT2
Organism
Homo sapiens (Human)
Status
Functioni
Synthesizes the galactose-alpha(1,3)-galactose group on the glycosphingolipid isoglobotrihexosylceramide or isogloboside 3 (iGb3) by catalyzing the transfer of galactose from UDP-Galactose to its acceptor molecule Gal-beta-1,4-Glc-ceramide. Can also catalyze the addition of galactose to iGb3 itself to form polygalactose structures.
1 PublicationCaution
According to a report, the spliced A3GALT2 mRNA is not detected in human tissues (PubMed:18630988). The functional activity of human A3GALT2 tested by expressing a chimeric protein containing the catalytic domain of human A3GALT2 is unable to synthesize isogloboside 3 (iGb3). Futhermore mutagenesis experiments in rat, show that the mutant 'Asn-253' of human A3GALT2 completely eliminates alpha-1,3-galactosyltransferase activity (PubMed:18630988).1 Publication
Catalytic activityi
- a β-D-galactosyl-(1→4)-N-acetyl-β-D-glucosaminyl derivative + UDP-α-D-galactose = an α-D-galactosyl-(1→3)-β-D-galactosyl-(1→4)-N-acetyl-β-D-glucosaminyl derivative + H+ + UDPBy similarityEC:2.4.1.87By similarityThis reaction proceeds in the forwardBy similarity direction.
- a β-D-Gal-(1→4)-β-D-Glc-(1↔1)-Cer(d18:1(4E)) + UDP-α-D-galactose = H+ + isogloboside iGb3Cer (d18:1(4E)) + UDPBy similarityThis reaction proceeds in the forwardBy similarity direction.
- This reaction proceeds in the forwardBy similarity direction.
Cofactori
Mn2+By similarity
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 199 | ManganeseBy similarity | 1 | |
Metal bindingi | 201 | ManganeseBy similarity | 1 |
GO - Molecular functioni
- glycosyltransferase activity Source: GO_Central
- metal ion binding Source: UniProtKB-KW
- N-acetyllactosaminide 3-alpha-galactosyltransferase activity Source: UniProtKB
GO - Biological processi
- carbohydrate metabolic process Source: InterPro
- cellular response to manganese ion Source: Ensembl
- glycosphingolipid biosynthetic process Source: Ensembl
- lipid glycosylation Source: GO_Central
Keywordsi
Molecular function | Glycosyltransferase, Transferase |
Biological process | Lipid metabolism |
Ligand | Manganese, Metal-binding |
Enzyme and pathway databases
PathwayCommonsi | U3KPV4 |
SignaLinki | U3KPV4 |
Names & Taxonomyi
Protein namesi | Recommended name: Alpha-1,3-galactosyltransferase 2Imported (EC:2.4.1.87By similarity)Alternative name(s): Isoglobotriaosylceramide synthase Short name: iGb3 synthase1 Publication Short name: iGb3S1 Publication |
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:30005, A3GALT2 |
neXtProti | NX_U3KPV4 |
VEuPathDBi | HostDB:ENSG00000184389 |
Subcellular locationi
Golgi apparatus
- Golgi stack membrane By similarity; Single-pass type II membrane protein By similarity
Note: Also found in numerous large vesicles throughout the cytoplasm of the soma.By similarity
Golgi apparatus
- Golgi apparatus Source: GO_Central
- Golgi cisterna membrane Source: UniProtKB-SubCell
Other locations
- integral component of membrane Source: UniProtKB-KW
- vesicle Source: GO_Central
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Topological domaini | 1 – 12 | CytoplasmicCuratedAdd BLAST | 12 | |
Transmembranei | 13 – 32 | Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST | 20 | |
Topological domaini | 33 – 340 | LumenalCuratedAdd BLAST | 308 |
Keywords - Cellular componenti
Golgi apparatus, MembranePathology & Biotechi
Organism-specific databases
DisGeNETi | 127550 |
OpenTargetsi | ENSG00000184389 |
Miscellaneous databases
Pharosi | U3KPV4, Tdark |
Genetic variation databases
BioMutai | A3GALT2 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000436524 | 1 – 340 | Alpha-1,3-galactosyltransferase 2Add BLAST | 340 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Glycosylationi | 58 | N-linked (GlcNAc...) asparagineSequence analysis | 1 | |
Glycosylationi | 100 | N-linked (GlcNAc...) asparagineSequence analysis | 1 |
Keywords - PTMi
GlycoproteinProteomic databases
MassIVEi | U3KPV4 |
PeptideAtlasi | U3KPV4 |
PRIDEi | U3KPV4 |
PTM databases
GlyGeni | U3KPV4, 2 sites |
Expressioni
Tissue specificityi
Expressed in thymus and monocyte derived dendritic cells.1 Publication
Gene expression databases
Bgeei | ENSG00000184389, Expressed in blood and 63 other tissues |
Organism-specific databases
HPAi | ENSG00000184389, Not detected |
Interactioni
Protein-protein interaction databases
STRINGi | 9606.ENSP00000475261 |
Miscellaneous databases
RNActi | U3KPV4, protein |
Family & Domainsi
Domaini
The conserved DXD motif is involved in cofactor binding. The manganese ion interacts with the beta-phosphate group of UDP and may also have a role in catalysis (By similarity).By similarity
Sequence similaritiesi
Belongs to the glycosyltransferase 6 family.Curated
Keywords - Domaini
Signal-anchor, Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | ENOG502QW2H, Eukaryota |
GeneTreei | ENSGT00950000182858 |
HOGENOMi | CLU_062445_1_0_1 |
OMAi | LLLYWFP |
OrthoDBi | 1204439at2759 |
PhylomeDBi | U3KPV4 |
Family and domain databases
Gene3Di | 3.90.550.10, 1 hit |
InterProi | View protein in InterPro IPR005076, Glyco_trans_6 IPR029044, Nucleotide-diphossugar_trans |
PANTHERi | PTHR10462, PTHR10462, 1 hit |
Pfami | View protein in Pfam PF03414, Glyco_transf_6, 1 hit |
SUPFAMi | SSF53448, SSF53448, 1 hit |
i Sequence
Sequence statusi: Complete.
U3KPV4-1 [UniParc]FASTAAdd to basket
10 20 30 40 50
MALKEGLRAW KRIFWRQILL TLGLLGLFLY GLPKFRHLEA LIPMGVCPSA
60 70 80 90 100
TMSQLRDNFT GALRPWARPE VLTCTPWGAP IIWDGSFDPD VAKQEARQQN
110 120 130 140 150
LTIGLTIFAV GRYLEKYLER FLETAEQHFM AGQSVMYYVF TELPGAVPRV
160 170 180 190 200
ALGPGRRLPV ERVARERRWQ DVSMARMRTL HAALGGLPGR EAHFMFCMDV
210 220 230 240 250
DQHFSGTFGP EALAESVAQL HSWHYHWPSW LLPFERDAHS AAAMAWGQGD
260 270 280 290 300
FYNHAAVFGG SVAALRGLTA HCAGGLDWDR ARGLEARWHD ESHLNKFFWL
310 320 330 340
HKPAKVLSPE FCWSPDIGPR AEIRRPRLLW APKGYRLLRN
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AL513327 Genomic DNA No translation available. |
CCDSi | CCDS60080.1 |
RefSeqi | NP_001073907.1, NM_001080438.1 |
Genome annotation databases
Ensembli | ENST00000442999; ENSP00000475261; ENSG00000184389 |
GeneIDi | 127550 |
KEGGi | hsa:127550 |
MANE-Selecti | ENST00000442999.3; ENSP00000475261.1; NM_001080438.1; NP_001073907.1 |
UCSCi | uc031plq.1, human |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AL513327 Genomic DNA No translation available. |
CCDSi | CCDS60080.1 |
RefSeqi | NP_001073907.1, NM_001080438.1 |
3D structure databases
SMRi | U3KPV4 |
ModBasei | Search... |
Protein-protein interaction databases
STRINGi | 9606.ENSP00000475261 |
PTM databases
GlyGeni | U3KPV4, 2 sites |
Genetic variation databases
BioMutai | A3GALT2 |
Proteomic databases
MassIVEi | U3KPV4 |
PeptideAtlasi | U3KPV4 |
PRIDEi | U3KPV4 |
Protocols and materials databases
Antibodypediai | 76335, 3 antibodies from 1 providers |
DNASUi | 127550 |
Genome annotation databases
Ensembli | ENST00000442999; ENSP00000475261; ENSG00000184389 |
GeneIDi | 127550 |
KEGGi | hsa:127550 |
MANE-Selecti | ENST00000442999.3; ENSP00000475261.1; NM_001080438.1; NP_001073907.1 |
UCSCi | uc031plq.1, human |
Organism-specific databases
CTDi | 127550 |
DisGeNETi | 127550 |
GeneCardsi | A3GALT2 |
HGNCi | HGNC:30005, A3GALT2 |
HPAi | ENSG00000184389, Not detected |
neXtProti | NX_U3KPV4 |
OpenTargetsi | ENSG00000184389 |
VEuPathDBi | HostDB:ENSG00000184389 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | ENOG502QW2H, Eukaryota |
GeneTreei | ENSGT00950000182858 |
HOGENOMi | CLU_062445_1_0_1 |
OMAi | LLLYWFP |
OrthoDBi | 1204439at2759 |
PhylomeDBi | U3KPV4 |
Enzyme and pathway databases
PathwayCommonsi | U3KPV4 |
SignaLinki | U3KPV4 |
Miscellaneous databases
BioGRID-ORCSi | 127550, 6 hits in 952 CRISPR screens |
GenomeRNAii | 127550 |
Pharosi | U3KPV4, Tdark |
PROi | PR:U3KPV4 |
RNActi | U3KPV4, protein |
Gene expression databases
Bgeei | ENSG00000184389, Expressed in blood and 63 other tissues |
Family and domain databases
Gene3Di | 3.90.550.10, 1 hit |
InterProi | View protein in InterPro IPR005076, Glyco_trans_6 IPR029044, Nucleotide-diphossugar_trans |
PANTHERi | PTHR10462, PTHR10462, 1 hit |
Pfami | View protein in Pfam PF03414, Glyco_transf_6, 1 hit |
SUPFAMi | SSF53448, SSF53448, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | A3LT2_HUMAN | |
Accessioni | U3KPV4Primary (citable) accession number: U3KPV4 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | July 6, 2016 |
Last sequence update: | November 13, 2013 | |
Last modified: | February 23, 2022 | |
This is version 49 of the entry and version 1 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
Reference proteomeDocuments
- Human chromosome 1
Human chromosome 1: entries, gene names and cross-references to MIM - SIMILARITY comments
Index of protein domains and families