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beta-1,3-glucuronyltransferase 3

Summary

Gene Symbol

B3GAT3

Aliases

GlcAT-I
galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3
glucuronosyltransferase I

Organism

Homo sapiens (human)

External Links

NCBI Gene

26229

GGDB ID

gg155

HGNC

923

mRNA

NM_012200

map

11q12.3

Protein

NP_036332

OMIM

606374

KEGG Gene ID

hsa:26229

PubChem

26229

Alliance of Genome Resources

HGNC:923

Annotation

Keyword

3D-structure
Alternative splicing
Disease variant
Disulfide bond
Glycoprotein
Golgi apparatus
Manganese
Metal-binding
Proteomics identification
Reference proteome
Signal-anchor
Transferase
Transmembrane helix

Proteins

Displaying **all 3** entries
UniProt	Protein Name
G3V150
Q5U676
O94766	Beta-1,3-glucuronyltransferase 3 Glucuronosyltransferase I UDP-GlcUA:Gal beta-1,3-Gal-R glucuronyltransferase

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **1 - 5** of 8 in total

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GO Term	Evidence Code	PMID
carbohydrate metabolic process	IBA
heparan sulfate proteoglycan biosynthetic process	IDA IMP	21763480 25893793
positive regulation of catalytic activity	IDA	24425863
chondroitin sulfate proteoglycan biosynthetic process	IBA IDA IMP	21763480 25893793
glycosaminoglycan biosynthetic process	IDA IMP NAS	10842173 24425863 25893793

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

organonitrogen compound metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan biosynthetic process

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan biosynthetic process [inference]

chondroitin sulfate proteoglycan metabolic process [inference]

chondroitin sulfate proteoglycan biosynthetic process

dermatan sulfate proteoglycan metabolic process [inference]

dermatan sulfate proteoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

protein modification process [inference]

protein glycosylation

organonitrogen compound biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

biosynthetic process [inference]

cellular biosynthetic process [inference]

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

sulfur compound biosynthetic process [inference]

heparan sulfate proteoglycan biosynthetic process

organic substance biosynthetic process [inference]

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

carbohydrate derivative biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

organonitrogen compound biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

cellular metabolic process [inference]

sulfur compound metabolic process [inference]

sulfur compound biosynthetic process [inference]

heparan sulfate proteoglycan biosynthetic process

cellular biosynthetic process [inference]

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

sulfur compound biosynthetic process [inference]

heparan sulfate proteoglycan biosynthetic process

primary metabolic process [inference]

carbohydrate metabolic process

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan biosynthetic process [inference]

chondroitin sulfate proteoglycan metabolic process [inference]

chondroitin sulfate proteoglycan biosynthetic process

dermatan sulfate proteoglycan metabolic process [inference]

dermatan sulfate proteoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

protein modification process [inference]

protein glycosylation

glycosylation [inference]

macromolecule glycosylation [inference]

protein glycosylation

organic substance metabolic process [inference]

carbohydrate metabolic process

macromolecule metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan biosynthetic process

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan biosynthetic process [inference]

chondroitin sulfate proteoglycan metabolic process [inference]

chondroitin sulfate proteoglycan biosynthetic process

dermatan sulfate proteoglycan metabolic process [inference]

dermatan sulfate proteoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

protein modification process [inference]

protein glycosylation

macromolecule modification [inference]

protein modification process [inference]

protein glycosylation

macromolecule glycosylation [inference]

protein glycosylation

carbohydrate derivative metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan biosynthetic process [inference]

chondroitin sulfate proteoglycan metabolic process [inference]

chondroitin sulfate proteoglycan biosynthetic process

dermatan sulfate proteoglycan metabolic process [inference]

dermatan sulfate proteoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

carbohydrate derivative biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

organonitrogen compound metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan biosynthetic process

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan biosynthetic process [inference]

chondroitin sulfate proteoglycan metabolic process [inference]

chondroitin sulfate proteoglycan biosynthetic process

dermatan sulfate proteoglycan metabolic process [inference]

dermatan sulfate proteoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

protein modification process [inference]

protein glycosylation

organonitrogen compound biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

organic substance biosynthetic process [inference]

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

carbohydrate derivative biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

organonitrogen compound biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

cellular process [inference]

cellular metabolic process [inference]

sulfur compound metabolic process [inference]

sulfur compound biosynthetic process [inference]

heparan sulfate proteoglycan biosynthetic process

cellular biosynthetic process [inference]

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

sulfur compound biosynthetic process [inference]

heparan sulfate proteoglycan biosynthetic process

biological regulation [inference]

regulation of biological process [inference]

regulation of localization [inference]

regulation of transport [inference]

regulation of intracellular transport [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of transport [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

regulation of protein transport [inference]

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

regulation of cellular localization [inference]

regulation of intracellular transport [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

regulation of protein localization [inference]

regulation of establishment of protein localization [inference]

regulation of protein transport [inference]

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein localization [inference]

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of biological process [inference]

positive regulation of cellular process [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein localization [inference]

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of transport [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

regulation of cellular process [inference]

positive regulation of cellular process [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein localization [inference]

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

regulation of cellular localization [inference]

regulation of intracellular transport [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

regulation of protein localization [inference]

regulation of establishment of protein localization [inference]

regulation of protein transport [inference]

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein localization [inference]

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

regulation of molecular function [inference]

positive regulation of molecular function [inference]

positive regulation of catalytic activity

regulation of catalytic activity [inference]

positive regulation of catalytic activity

Displaying **all 5** entries
GO Term	Evidence Code	PMID
cis-Golgi network	IDA	21763480 25893793
membrane	HDA NAS	10842173 19946888
Golgi apparatus	IDA	21763480
extracellular exosome	HDA	19199708
Golgi membrane	IBA TAS

GO Hierarchy

cellular component

cellular anatomical entity [inference]

membrane

organelle membrane [inference]

bounding membrane of organelle [inference]

Golgi membrane

organelle [inference]

membrane-bounded organelle [inference]

vesicle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular membrane-bounded organelle [inference]

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

extracellular organelle [inference]

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

Displaying **all 5** entries
GO Term	Evidence Code	PMID
protein phosphatase activator activity	IDA	24425863
galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity	IBA NAS	10842173
metal ion binding	IEA
protein binding	IPI	24425863 32707033
glucuronosyltransferase activity	IDA IMP	21763480 25893793

GO Hierarchy

molecular function

catalytic activity [inference]

transferase activity [inference]

glycosyltransferase activity [inference]

UDP-glycosyltransferase activity [inference]

glucuronosyltransferase activity

galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity

hexosyltransferase activity [inference]

glucuronosyltransferase activity

galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity

binding [inference]

protein binding

ion binding [inference]

cation binding [inference]

metal ion binding

molecular function regulator activity [inference]

enzyme regulator activity [inference]

enzyme activator activity [inference]

phosphatase activator activity [inference]

protein phosphatase activator activity

phosphatase regulator activity [inference]

phosphatase activator activity [inference]

protein phosphatase activator activity

protein phosphatase regulator activity [inference]

protein phosphatase activator activity

molecular function activator activity [inference]

enzyme activator activity [inference]

phosphatase activator activity [inference]

protein phosphatase activator activity

Gene Ontology information from NCBI Gene and UniProt.

Human Protein Atlas

ENSG00000149541

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

GlycoGene Database (GGDB)

GGDB ID

gg155

Gene Symbol

B3GAT3

Reactions

Displaying 1 entry
Donor	Acceptor	Product	Reference
UDP-GlcUA	G87877ES	G14371GV	9506957

Displaying 1 entry
Donor	Acceptor	Product	Reference
UDP-GlcUA	G87877ES	G14371GV	9506957

Displaying **all 2** entries
Donor	Acceptor	Product	Reference
UDP-GlcUA	G95036CM		9506957
UDP-GlcUA	G40609EP		9506957

Orthologous Gene

Displaying **all 3** entries
Species	Protein	mRNA
Rattus norvegicus	XP_238155	XM_238155
Drosophila melanogaster	NP_726910	NM_167003
Mus musculus	NP_077218	NM_024256

KEGG BRITE Database

Orthology

K10158

Name

galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3 [EC:2.4.1.135]

References

Reactions

Displaying 1 entry
KEGG Reaction	Enzyme	Acceptor	Product
R05928	2.4.1.135	G87877ES	G14371GV

Rhea

RHEA:56508

UDP-α-D-glucuronate

H⁺ + UDP

Enzyme

2.4.1.135

PMID

18400750

RHEA:24168

UDP-α-D-glucuronate

H⁺ + UDP

Enzyme

2.4.1.135

PMID

Disease

Disease Ontology

Displaying entries **21 - 30** of 79 in total

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DO ID	Disease Name	Source
DOID:0080333	aortic valve disease 1	DisGeNET
DOID:0080334	aortic valve disease 2	DisGeNET
DOID:0080575	Larsen-like syndrome B3GAT3 type	Glyco-Disease Genes Database DisGeNET Alliance of Genome Resources
DOID:0110106	atrial heart septal defect 1	DisGeNET
DOID:0110107	atrial heart septal defect 2	DisGeNET
DOID:0110108	atrial heart septal defect 3	DisGeNET
DOID:0110109	atrial heart septal defect 4	DisGeNET
DOID:0110110	atrial heart septal defect 5	DisGeNET
DOID:0110111	atrial heart septal defect 6	DisGeNET
DOID:0110112	atrial heart septal defect 7	DisGeNET

The Human Phenotype Ontology

Displaying entries **41 - 50** of 81 in total

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HPO ID	HPO Term
HP:0001631	Atrial septal defect
HP:0001634	Mitral valve prolapse
HP:0001640	Cardiomegaly
HP:0001647	Bicuspid aortic valve
HP:0001655	Patent foramen ovale
HP:0001712	Left ventricular hypertrophy
HP:0001762	Talipes equinovarus
HP:0001763	Pes planus
HP:0001772	Talipes equinovalgus
HP:0001822	Hallux valgus

Displaying 1 entry
Disease ID	Disease Name
OMIM:245600	Larsen-like syndrome, B3GAT3 type

PubChem Disease

MedGen Diseases

Larsen-like syndrome, B3GAT3 type

OMIM Phenotypes

MULTIPLE JOINT DISLOCATIONS, SHORT STATURE, AND CRANIOFACIAL DYSMORPHISM WITH OR WITHOUT CONGENITAL HEART DEFECTS; JDSCD

KEGG Diseases

Multiple joint dislocations, short stature, craniofacial dysmorphism, and congenital heart defects

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

Ortholog

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Caenorhabditis elegans	174517	WB:WBGene00005026
Caenorhabditis elegans	184025	WB:WBGene00008479
Drosophila melanogaster	251900	FB:FBgn0066114
Ciona intestinalis	778533		CIOIN15244
Callorhinchus milii	103171591		CALMI45788
Latimeria chalumnae	102364764		LATCH18458
Danio rerio	192334	ZFIN:ZDB-GENE-020419-3	DANRE39848
Astyanax mexicanus	103028547		ASTMX05626
Ictalurus punctatus	108268305		ICTPU32895
Electrophorus electricus	113579800		ELEEL24862

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

All Resources

Other Information

Tools

Search Tools

Summary

External Links

Annotation

GlycoGene Database (GGDB)

KEGG BRITE Database

PubChem Disease

International Collaboration

Acknowledgements