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Q9P2W7 (B3GA1_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 133. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (7) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Web links·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 1

EC=2.4.1.135
Alternative name(s):
Beta-1,3-glucuronyltransferase 1
Glucuronosyltransferase P
Short name=GlcAT-P
UDP-GlcUA:glycoprotein beta-1,3-glucuronyltransferase
Short name=GlcUAT-P
Gene names
Name:B3GAT1
Synonyms:GLCATP
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length334 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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 similarity.

Catalytic activity

UDP-glucuronate + 3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosylprotein = UDP + 3-beta-D-glucuronosyl-3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosylprotein.

Cofactor

Manganese.

Pathway

Protein modification; protein glycosylation.

Subunit structure

Homodimer Potential.

Subcellular location

Golgi apparatus membrane; Single-pass type II membrane protein.

Tissue specificity

Mainly expressed in the brain.

Sequence similarities

Belongs to the glycosyltransferase 43 family.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 334334Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 1
PRO_0000195167

Regions

Topological domain1 – 66Cytoplasmic Potential
Transmembrane7 – 2721Helical; Signal-anchor for type II membrane protein; Potential
Topological domain28 – 334307Lumenal Potential
Nucleotide binding91 – 933UDP-glucuronate binding
Nucleotide binding195 – 1973UDP-glucuronate binding
Nucleotide binding311 – 3133UDP-glucuronate binding
Region245 – 25410Interaction with galactose moiety of substrate glycoprotein

Sites

Active site2841Proton donor/acceptor
Metal binding1971Manganese By similarity
Binding site1221UDP-glucuronate
Binding site1651UDP-glucuronate
Binding site1701UDP-glucuronate By similarity
Site2281Interaction with galactose moiety of substrate glycoprotein
Site3211Interaction with galactose moiety of substrate glycoprotein By similarity

Amino acid modifications

Modified residue1031Phosphothreonine By similarity
Modified residue1081Phosphothreonine By similarity
Glycosylation1401N-linked (GlcNAc...) Potential
Glycosylation1841N-linked (GlcNAc...) Potential
Glycosylation3031N-linked (GlcNAc...) Potential

Natural variations

Natural variant1311A → T.
Corresponds to variant rs35434644 [ dbSNP | Ensembl ].
VAR_044538

Experimental info

Sequence conflict2401G → R in BAA96077. Ref.1

Secondary structure

................................................. 334
Helix Strand Turn

Details...

Sequences

Sequence LengthMass (Da)Tools
Q9P2W7 [UniParc].

Last modified July 22, 2008. Version 2.
Checksum: 0DF42399D19701B3

FASTA33438,256
        10         20         30         40         50         60 
MPKRRDILAI VLIVLPWTLL ITVWHQSTLA PLLAVHKDEG SDPRRETPPG ADPREYCTSD 

        70         80         90        100        110        120 
RDIVEVVRTE YVYTRPPPWS DTLPTIHVVT PTYSRPVQKA ELTRMANTLL HVPNLHWLVV 

       130        140        150        160        170        180 
EDAPRRTPLT ARLLRDTGLN YTHLHVETPR NYKLRGDARD PRIPRGTMQR NLALRWLRET 

       190        200        210        220        230        240 
FPRNSSQPGV VYFADDDNTY SLELFEEMRS TRRVSVWPVA FVGGLRYEAP RVNGAGKVVG 

       250        260        270        280        290        300 
WKTVFDPHRP FAIDMAGFAV NLRLILQRSQ AYFKLRGVKG GYQESSLLRE LVTLNDLEPK 

       310        320        330 
AANCTKILVW HTRTEKPVLV NEGKKGFTDP SVEI 

« Hide

References

« Hide 'large scale' references
[1]"Cloning and chromosomal mapping of human glucuronyltransferase involved in biosynthesis of the HNK-1 carbohydrate epitope."
Mitsumoto Y., Oka S., Sakuma H., Inazawa J., Kawasaki T.
Genomics 65:166-173(2000) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
Tissue: Brain.
[2]"Cloning of human full open reading frames in Gateway(TM) system entry vector (pDONR201)."
Ebert L., Schick M., Neubert P., Schatten R., Henze S., Korn B.
Submitted (JUN-2004) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
[3]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
Tissue: Brain.
[4]"Structural basis for acceptor substrate recognition of a human glucuronyltransferase, GlcAT-P, an enzyme critical in the biosynthesis of the carbohydrate epitope HNK-1."
Kakuda S., Shiba T., Ishiguro M., Tagawa H., Oka S., Kajihara Y., Kawasaki T., Wakatsuki S., Kato R.
J. Biol. Chem. 279:22693-22703(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: X-RAY CRYSTALLOGRAPHY (1.85 ANGSTROMS) OF 83-334 IN COMPLEX WITH UDP-GLUCURONIC ACID AND GALACTOSE MOIETY OF SUBSTRATE GLYCOPROTEIN.
+Additional computationally mapped references.

Web resources

GGDB

GlycoGene database

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AB029396 mRNA. Translation: BAA96077.1.
CR457098 mRNA. Translation: CAG33379.1.
BC010466 mRNA. Translation: AAH10466.1.
RefSeqNP_061114.2. NM_018644.3.
NP_473366.1. NM_054025.2.
UniGeneHs.381050.

3D structure databases

PDBe
RCSB PDB
PDBj
EntryMethodResolution (Å)ChainPositionsPDBsum
1V82X-ray1.85A/B83-334[»]
1V83X-ray1.90A/B83-334[»]
1V84X-ray1.82A/B83-334[»]
ProteinModelPortalQ9P2W7.
SMRQ9P2W7. Positions 83-334.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid117990. 2 interactions.
IntActQ9P2W7. 2 interactions.
STRING9606.ENSP00000307875.

Protein family/group databases

CAZyGT43. Glycosyltransferase Family 43.

PTM databases

PhosphoSiteQ9P2W7.

Polymorphism databases

DMDM205830910.

Proteomic databases

PaxDbQ9P2W7.
PRIDEQ9P2W7.

Protocols and materials databases

DNASU27087.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000312527; ENSP00000307875; ENSG00000109956.
ENST00000392580; ENSP00000376359; ENSG00000109956.
ENST00000524765; ENSP00000433847; ENSG00000109956.
GeneID27087.
KEGGhsa:27087.
UCSCuc001qhq.3. human.

Organism-specific databases

CTD27087.
GeneCardsGC11M134281.
H-InvDBHIX0010301.
HGNCHGNC:921. B3GAT1.
HPACAB002500.
CAB010893.
MIM151290. gene.
neXtProtNX_Q9P2W7.
PharmGKBPA25215.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG310844.
HOVERGENHBG050650.
InParanoidQ9P2W7.
KOK00735.
OrthoDBEOG7QG44X.
PhylomeDBQ9P2W7.
TreeFamTF313522.

Enzyme and pathway databases

BioCycMetaCyc:HS03272-MONOMER.
BRENDA2.4.1.135. 2681.
ReactomeREACT_111217. Metabolism.
REACT_116125. Disease.
UniPathwayUPA00378.

Gene expression databases

ArrayExpressQ9P2W7.
BgeeQ9P2W7.
CleanExHS_B3GAT1.
GenevestigatorQ9P2W7.

Family and domain databases

InterProIPR005027. Glyco_trans_43.
[Graphical view]
PANTHERPTHR10896. PTHR10896. 1 hit.
PfamPF03360. Glyco_transf_43. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

EvolutionaryTraceQ9P2W7.
GeneWikiB3GAT1.
GenomeRNAi27087.
NextBio49699.
PROQ9P2W7.
SOURCESearch...

Entry information

Entry nameB3GA1_HUMAN
AccessionPrimary (citable) accession number: Q9P2W7
Secondary accession number(s): Q96FS7
Entry history
Integrated into UniProtKB/Swiss-Prot: June 1, 2001
Last sequence update: July 22, 2008
Last modified: April 16, 2014
This is version 133 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

PDB cross-references

Index of Protein Data Bank (PDB) cross-references

PATHWAY comments

Index of metabolic and biosynthesis pathways

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 11

Human chromosome 11: entries, gene names and cross-references to MIM