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Protein

Beta-1,4-glucuronyltransferase 1

Gene

B4gat1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Beta-1,4-glucuronyltransferase involved in O-mannosylation of alpha-dystroglycan (DAG1). Transfers a glucuronic acid (GlcA) residue onto a xylose (Xyl) acceptor to produce the glucuronyl-beta-1,4-xylose-beta disaccharide primer, which is further elongated by LARGE1, during synthesis of phosphorylated O-mannosyl glycan. Phosphorylated O-mannosyl glycan is a carbohydrate is a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity (PubMed:25279699). Required for axon guidance; via its function in O-mannosylation of alpha-dystroglycan (DAG1) (PubMed:23217742).2 Publications

Cofactori

Mn2+By similarity

Pathwayi: protein glycosylation

This protein is involved in the pathway protein glycosylation, which is part of Protein modification.1 Publication
View all proteins of this organism that are known to be involved in the pathway protein glycosylation and in Protein modification.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi227ManganeseBy similarity1
Metal bindingi229ManganeseBy similarity1

GO - Molecular functioni

GO - Biological processi

  • axon guidance Source: MGI
  • protein glycosylation Source: MGI
  • protein O-linked mannosylation Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Glycosyltransferase, Transferase

Keywords - Ligandi

Manganese, Metal-binding

Enzyme and pathway databases

ReactomeiR-MMU-2022854. Keratan sulfate biosynthesis.
R-MMU-5173105. O-linked glycosylation.
UniPathwayiUPA00378.

Protein family/group databases

CAZyiGT49. Glycosyltransferase Family 49.

Names & Taxonomyi

Protein namesi
Recommended name:
Beta-1,4-glucuronyltransferase 1By similarity (EC:2.4.1.-By similarity)
Alternative name(s):
I-beta-1,3-N-acetylglucosaminyltransferase
Short name:
iGnT
N-acetyllactosaminide beta-1,3-N-acetylglucosaminyltransferase
Poly-N-acetyllactosamine extension enzyme
UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1
Gene namesi
Name:B4gat1By similarity
Synonyms:B3gnt1, B3gnt6
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 19

Organism-specific databases

MGIiMGI:1919680. B4gat1.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 8CytoplasmicSequence analysis8
Transmembranei9 – 36Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST28
Topological domaini37 – 415LumenalSequence analysisAdd BLAST379

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Golgi apparatus, Membrane

Pathology & Biotechi

Disruption phenotypei

Early embryonic lethality.1 Publication

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi155M → T: Mild muscular dystrophy phenotype of variable penetrance due to defects in axon guidance. Mislocalization to the endoplasmic reticulum. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000805561 – 415Beta-1,4-glucuronyltransferase 1Add BLAST415

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi204N-linked (GlcNAc...)Sequence analysis1
Glycosylationi300N-linked (GlcNAc...)Sequence analysis1

Keywords - PTMi

Glycoprotein

Proteomic databases

PaxDbiQ8BWP8.
PeptideAtlasiQ8BWP8.
PRIDEiQ8BWP8.

PTM databases

PhosphoSitePlusiQ8BWP8.

Expressioni

Gene expression databases

BgeeiENSMUSG00000047379.
GenevisibleiQ8BWP8. MM.

Interactioni

Subunit structurei

Interacts with LARGE1 and LARGE2.By similarity

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000062016.

Structurei

3D structure databases

ProteinModelPortaliQ8BWP8.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the glycosyltransferase 49 family.Curated

Keywords - Domaini

Signal-anchor, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3765. Eukaryota.
ENOG410XRNY. LUCA.
GeneTreeiENSGT00530000063165.
HOGENOMiHOG000064521.
HOVERGENiHBG080885.
InParanoidiQ8BWP8.
KOiK00741.
OMAiMPMSKTE.
OrthoDBiEOG091G0K0J.
PhylomeDBiQ8BWP8.
TreeFamiTF319168.

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q8BWP8-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MQMSYAIRCA FYQLLLAALM LVAMLQLLYL SLLSGLHGQE EQEQYFEFFP
60 70 80 90 100
PSPRSVDQVK SQLRTALASG GVLDASGDYR VYRGLLKTTM DPNDVILATH
110 120 130 140 150
ASVDNLLHLS GLLERWEGPL SVSVFAATKE EAQLATVLAY ALSSHCPEMR
160 170 180 190 200
ARVAMHLVCP SRYEAAVPDP REPGEFALLR SCQEVFDKLA RVAQPGINYA
210 220 230 240 250
LGTNTSYPNN LLRNLAREEA NYALVIDVDM VPSEGLWRGL REMLDQSNHW
260 270 280 290 300
DGTALVVPAF EIRRSRRMPM NKNELVQLYQ VGEVRPFYYG LCTPCHAPTN
310 320 330 340 350
YSRWVNLPEE SLLRPAYVVP WRDPWEPFYV AGGKVPTFDE RFRQYGFNRI
360 370 380 390 400
SQACELHVAG FNFEVLNEGF LVHKGFKEAL KFHPQKEAEN QRNKILYRQF
410
KQELKARYPN SPHRC
Length:415
Mass (Da):47,384
Last modified:March 1, 2003 - v1
Checksum:i7B74BEB591EAA4DC
GO
Isoform 2 (identifier: Q8BWP8-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     353-379: ACELHVAGFNFEVLNEGFLVHKGFKEA → VLKRERACRTKTGGVSGVKWPLVERRQ
     380-415: Missing.

Note: No experimental confirmation available.
Show »
Length:379
Mass (Da):43,009
Checksum:iF1E39CBDF0D3CF27
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti175 – 176EF → RV in AAH02191 (PubMed:15489334).Curated2

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_014004353 – 379ACELH…GFKEA → VLKRERACRTKTGGVSGVKW PLVERRQ in isoform 2. 1 PublicationAdd BLAST27
Alternative sequenceiVSP_014005380 – 415Missing in isoform 2. 1 PublicationAdd BLAST36

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK050347 mRNA. Translation: BAC34203.1.
AK083857 mRNA. Translation: BAC39041.1.
AK158899 mRNA. Translation: BAE34720.1.
BC002191 mRNA. Translation: AAH02191.1.
BC069927 mRNA. Translation: AAH69927.1.
CCDSiCCDS29446.1. [Q8BWP8-1]
RefSeqiNP_780592.1. NM_175383.2. [Q8BWP8-1]
UniGeneiMm.491160.

Genome annotation databases

EnsembliENSMUST00000053705; ENSMUSP00000062016; ENSMUSG00000047379. [Q8BWP8-1]
GeneIDi108902.
KEGGimmu:108902.
UCSCiuc008gbx.1. mouse. [Q8BWP8-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK050347 mRNA. Translation: BAC34203.1.
AK083857 mRNA. Translation: BAC39041.1.
AK158899 mRNA. Translation: BAE34720.1.
BC002191 mRNA. Translation: AAH02191.1.
BC069927 mRNA. Translation: AAH69927.1.
CCDSiCCDS29446.1. [Q8BWP8-1]
RefSeqiNP_780592.1. NM_175383.2. [Q8BWP8-1]
UniGeneiMm.491160.

3D structure databases

ProteinModelPortaliQ8BWP8.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000062016.

Protein family/group databases

CAZyiGT49. Glycosyltransferase Family 49.

PTM databases

PhosphoSitePlusiQ8BWP8.

Proteomic databases

PaxDbiQ8BWP8.
PeptideAtlasiQ8BWP8.
PRIDEiQ8BWP8.

Protocols and materials databases

DNASUi108902.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000053705; ENSMUSP00000062016; ENSMUSG00000047379. [Q8BWP8-1]
GeneIDi108902.
KEGGimmu:108902.
UCSCiuc008gbx.1. mouse. [Q8BWP8-1]

Organism-specific databases

CTDi11041.
MGIiMGI:1919680. B4gat1.

Phylogenomic databases

eggNOGiKOG3765. Eukaryota.
ENOG410XRNY. LUCA.
GeneTreeiENSGT00530000063165.
HOGENOMiHOG000064521.
HOVERGENiHBG080885.
InParanoidiQ8BWP8.
KOiK00741.
OMAiMPMSKTE.
OrthoDBiEOG091G0K0J.
PhylomeDBiQ8BWP8.
TreeFamiTF319168.

Enzyme and pathway databases

UniPathwayiUPA00378.
ReactomeiR-MMU-2022854. Keratan sulfate biosynthesis.
R-MMU-5173105. O-linked glycosylation.

Miscellaneous databases

PROiQ8BWP8.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000047379.
GenevisibleiQ8BWP8. MM.

Family and domain databases

ProtoNetiSearch...

Entry informationi

Entry nameiB4GA1_MOUSE
AccessioniPrimary (citable) accession number: Q8BWP8
Secondary accession number(s): Q3TY43, Q8BJH9, Q99LW7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: June 7, 2005
Last sequence update: March 1, 2003
Last modified: November 30, 2016
This is version 112 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.