Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3

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Reviewed, UniProtKB/Swiss-Prot O94766 (B3GA3_HUMAN)

Last modified November 4, 2008. Version 85. History...

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Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Sequence annotation (Features) · Sequences · References · Web resources · Cross-references · Entry information · Relevant documents

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Names and origin

Protein names

Recommended name:
    Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3
    EC=2.4.1.135
Alternative name(s):
    Beta-1,3-glucuronyltransferase 3
    Glucuronosyltransferase-I
      Short name=GlcAT-I
    UDP-GlcUA:Gal beta-1,3-Gal-R glucuronyltransferase
      Short name=GlcUAT-I

Gene names

Name:

B3GAT3

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

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Protein attributes

Sequence length	335 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is not processed.
Protein existence	Evidence at protein level.

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General annotation (Comments)

Function	Glycosaminoglycans biosynthesis. Involved in forming the linkage tetrasaccharide present in heparan sulfate and chondroitin sulfate. Transfers a glucuronic acid moiety from the uridine diphosphate-glucuronic acid (UDP-GlcUA) to the common linkage region trisaccharide Gal-beta-1,3-Gal-beta-1,4-Xyl covalently bound to a Ser residue at the glycosaminylglycan attachment site of proteoglycans. Can also play a role in the biosynthesis of l2/HNK-1 carbohydrate epitope on glycoproteins. Shows strict specificity for Gal-beta-1,3-Gal-beta-1,4-Xyl, exhibiting negligible incorporation into other galactoside substrates including Galbeta1-3Gal beta1-O-benzyl, Galbeta1-4GlcNAc and Galbeta1-4Glc.
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.
Enzyme regulation	Inhibited by EDTA.
Pathway	Protein modification; protein glycosylation.
Subunit structure	Homodimer; disulfide-linked.
Subcellular location	Golgi apparatus membrane; Single-pass type II membrane protein.
Tissue specificity	Ubiquitous (but weakly expressed in all tissues examined).
Post-translational modification	N-glycosylated.
Sequence similarities	Belongs to the glycosyltransferase 43 family.

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Ontologies

Keywords
Cellular component	Golgi apparatus Membrane
Domain	Signal-anchor Transmembrane
Ligand	Manganese Metal-binding
Molecular function	Transferase
PTM	Glycoprotein
Technical term	3D-structure
Gene Ontology (GO)
Biological process	glycosaminoglycan biosynthetic process Ref.2 Non-traceable author statement. Source: UniProtKB
Cellular component	Golgi membrane Non-traceable author statement. Source: UniProtKB integral to membrane Ref.2 Non-traceable author statement. Source: UniProtKB
Molecular function	galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity Ref.2 Non-traceable author statement. Source: UniProtKB manganese ion binding Non-traceable author statement. Source: UniProtKB
Complete GO annotation...

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Sequence annotation (Features)

Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Molecule processing

Chain

1 – 335

335

Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3

PRO_0000195176

Regions

Topological domain

1 – 7

Cytoplasmic Potential

Transmembrane

8 – 28

Signal-anchor for type II membrane protein Potential

Topological domain

29 – 335

307

Lumenal Potential

Sites

Active site

281

Proton acceptor

Metal binding

196

Manganese

Amino acid modifications

Glycosylation

300

N-linked (GlcNAc...)

Disulfide bond

Interchain

Experimental info

Mutagenesis

C → A: Loss of dimer formation and reduced activity

Mutagenesis

301

C → A: Enzyme inactivation and loss of glycosylation

Sequence conflict

204

F → S in BAA34537. Ref.1

Secondary structure

335

Helix Strand Turn

Details...

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Sequences

Sequence

Length

Mass (Da)

Tools

O94766-1 [UniParc].

Last modified April 26, 2004. Version 2.
Checksum: 5EC45408597F1C0F
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FASTA

335

37,122

        10         20         30         40         50         60 
MKLKLKNVFL AYFLVSIAGL LYALVQLGQP CDCLPPLRAA AEQLRQKDLR ISQLQAELRR 

        70         80         90        100        110        120 
PPPAPAQPPE PEALPTIYVV TPTYARLVQK AELVRLSQTL SLVPRLHWLL VEDAEGPTPL 

       130        140        150        160        170        180 
VSGLLAASGL LFTHLVVLTP KAQRLREGEP GWVHPRGVEQ RNKALDWLRG RGGAVGGEKD 

       190        200        210        220        230        240 
PPPPGTQGVV YFADDDNTYS RELFEEMRWT RGVSVWPVGL VGGLRFEGPQ VQDGRVVGFH 

       250        260        270        280        290        300 
TAWEPSRPFP VDMAGFAVAL PLLLDKPNAQ FDSTAPRGHL ESSLLSHLVD PKDLEPRAAN 

       310        320        330 
CTRVLVWHTR TEKPKMKQEE QLQRQGRGSD PAIEV

« Hide

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References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Molecular cloning and expression of glucuronyltransferase I involved in the biosynthesis of the glycosaminoglycan-protein linkage region of proteoglycans." Kitagawa H., Tone Y., Tamura J., Neumann K.W., Ogawa T., Oka S., Kawasaki T., Sugahara K. J. Biol. Chem. 273:6615-6618(1998) [PubMed: 9506957] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], CHARACTERIZATION. Tissue: Placenta.
[2]	"Structure/function of the human Ga1beta1,3-glucuronosyltransferase. Dimerization and functional activity are mediated by two crucial cysteine residues." Ouzzine M., Gulberti S., Netter P., Magdalou J., Fournel-Gigleux S. J. Biol. Chem. 275:28254-28260(2000) [PubMed: 10842173] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], CHARACTERIZATION, MUTAGENESIS.
[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: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Eye and Skin.
[4]	"Three proteins involved in Caenorhabditis elegans vulval invagination are similar to components of a glycosylation pathway." Herman T., Horvitz H.R. Proc. Natl. Acad. Sci. U.S.A. 96:974-979(1999) [PubMed: 9927678] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 9-335. Tissue: Brain.
[5]	"Characterization of recombinant human glucuronyltransferase I involved in the biosynthesis of the glycosaminoglycan-protein linkage region of proteoglycans." Tone Y., Kitagawa H., Imiya K., Oka S., Kawasaki T., Sugahara K. FEBS Lett. 459:415-420(1999) [PubMed: 10526176] [Abstract] Cited for: CHARACTERIZATION.
[6]	"Heparan/chondroitin sulfate biosynthesis. Structure and mechanism of human glucuronyltransferase I." Pedersen L.C., Tsuchida K., Kitagawa H., Sugahara K., Darden T.A., Negishi M. J. Biol. Chem. 275:34580-34585(2000) [PubMed: 10946001] [Abstract] Cited for: X-RAY CRYSTALLOGRAPHY (3.0 ANGSTROMS) OF 76-335. Tissue: Liver.
+	Additional computationally mapped references.

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Web resources

GGDB

GlycoGene database

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Cross-references

Sequence databases

AB009598 mRNA. Translation: BAA34537.1.
BC007906 mRNA. Translation: AAH07906.1.
BC071961 mRNA. Translation: AAH71961.1.
AJ005865 mRNA. Translation: CAA06742.1.

RefSeq

NP_036332.2.

UniGene

Hs.502759

3D structure databases

Entry	Method	Resolution (Å)	Chain	Positions	PDBsum
1FGG	X-ray	2.30	A/B	76-335	[»]
1KWS	X-ray	2.10	A/B	76-335	[»]
3CU0	X-ray	1.90	A/B	76-335	[»]

ModBase

Search...

PTM databases

PhosphoSite

O94766.

Genome annotation databases

Ensembl

ENSG00000149541. Homo sapiens. [Contig view]

GeneID

26229.

KEGG

hsa:26229.

Organism-specific databases

H-InvDB

HIX0009715.

HGNC

HGNC:923. B3GAT3.

MIM

606374. gene.

PharmGKB

PA25217.

GenAtlas

Search...

GeneCards

Search...

Phylogenomic databases

HOVERGEN

O94766.

Gene expression databases

CleanEx

HS_B3GAT3.

GermOnline

ENSG00000149541. Homo sapiens.

Family and domain databases

InterPro

IPR005027. Glyco_trans_43.
[Graphical view]

PANTHER

PTHR10896. Glyco_trans_43. 1 hit.

Pfam

PF03360. Glyco_transf_43. 1 hit.
[Graphical view]

ProtoNet

Search...

Other Resources

LinkHub

O94766.

NextBio

48391.

SOURCE

Search...

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Entry information

Entry name

B3GA3_HUMAN

Accession

Primary (citable) accession number: O94766
Secondary accession number(s): Q96I06, Q9UEP0

Entry history

Integrated into UniProtKB/Swiss-Prot:	June 1, 2001
Last sequence update:	April 26, 2004
Last modified:	November 4, 2008
This is version 85 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)

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Relevant documents

Human chromosome 11

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

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

Names and origin

Protein attributes

General annotation (Comments)

Ontologies

Keywords

Gene Ontology (GO)

Sequence annotation (Features)

Molecule processing

Regions

Sites

Amino acid modifications

Experimental info

Secondary structure

Sequences

References

Web resources

Cross-references

Sequence databases

3D structure databases

PTM databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Gene expression databases

Family and domain databases

Other Resources

Entry information

Relevant documents