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Protein

Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 2

Gene

B3GAT2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Involved in the biosynthesis of L2/HNK-1 carbohydrate epitope on both glycolipids and glycoproteins.By similarity

Catalytic activityi

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.

Cofactori

Pathway:iprotein glycosylation

This protein is involved in the pathway protein glycosylation, which is part of Protein modification.
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)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei118 – 1181UDP-glucuronateBy similarity
Binding sitei155 – 1551UDP-glucuronateBy similarity
Binding sitei160 – 1601UDP-glucuronateBy similarity
Metal bindingi187 – 1871ManganeseBy similarity
Sitei218 – 2181Interaction with galactose moiety of substrate glycoproteinBy similarity
Active sitei273 – 2731Proton donor/acceptorBy similarity
Sitei310 – 3101Interaction with galactose moiety of substrate glycoproteinBy similarity

Regions

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi87 – 893UDP-glucuronate bindingBy similarity
Nucleotide bindingi185 – 1873UDP-glucuronate bindingBy similarity
Nucleotide bindingi300 – 3023UDP-glucuronate bindingBy similarity

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Transferase

Keywords - Ligandi

Manganese, Metal-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS03557-MONOMER.
ReactomeiREACT_121408. A tetrasaccharide linker sequence is required for GAG synthesis.
UniPathwayiUPA00378.

Protein family/group databases

CAZyiGT43. Glycosyltransferase Family 43.

Names & Taxonomyi

Protein namesi
Recommended name:
Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 2 (EC:2.4.1.135)
Alternative name(s):
Beta-1,3-glucuronyltransferase 2
GlcAT-D
UDP-glucuronosyltransferase S
Short name:
GlcAT-S
Short name:
Glucuronosyltransferase S
Gene namesi
Name:B3GAT2
Synonyms:GLCATS, KIAA1963
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 6

Organism-specific databases

HGNCiHGNC:922. B3GAT2.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 22CytoplasmicSequence Analysis
Transmembranei3 – 2321Helical; Signal-anchor for type II membrane proteinSequence AnalysisAdd
BLAST
Topological domaini24 – 323300LumenalSequence AnalysisAdd
BLAST

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Golgi apparatus, Membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA25216.

Polymorphism and mutation databases

BioMutaiB3GAT2.
DMDMi14285363.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 323323Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 2PRO_0000195172Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi67 – 671N-linked (GlcNAc...)Sequence Analysis
Glycosylationi292 – 2921N-linked (GlcNAc...)Sequence Analysis

Keywords - PTMi

Glycoprotein

Proteomic databases

PaxDbiQ9NPZ5.
PRIDEiQ9NPZ5.

Expressioni

Tissue specificityi

Expressed in the trachea, retina, spinal cord, hippocampus and other brain regions, and, at lower levels, in testis and ovary.

Gene expression databases

BgeeiQ9NPZ5.
CleanExiHS_B3GAT2.
ExpressionAtlasiQ9NPZ5. baseline and differential.
GenevisibleiQ9NPZ5. HS.

Organism-specific databases

HPAiHPA012059.

Interactioni

Subunit structurei

Homodimer.Curated

Protein-protein interaction databases

STRINGi9606.ENSP00000230053.

Structurei

Secondary structure

1
323
Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Beta strandi82 – 887Combined sources
Helixi94 – 10512Combined sources
Beta strandi111 – 12111Combined sources
Helixi124 – 1329Combined sources
Beta strandi137 – 1415Combined sources
Helixi157 – 17014Combined sources
Beta strandi173 – 1753Combined sources
Beta strandi180 – 1834Combined sources
Helixi193 – 1986Combined sources
Beta strandi202 – 2065Combined sources
Beta strandi209 – 2124Combined sources
Beta strandi215 – 2239Combined sources
Beta strandi226 – 2316Combined sources
Helixi244 – 2463Combined sources
Beta strandi247 – 2504Combined sources
Helixi251 – 2566Combined sources
Beta strandi266 – 2683Combined sources
Helixi269 – 2713Combined sources
Helixi272 – 2787Combined sources
Helixi283 – 2853Combined sources
Helixi290 – 2934Combined sources
Beta strandi313 – 3153Combined sources

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
2D0JX-ray2.00A/B/C/D78-323[»]
ProteinModelPortaliQ9NPZ5.
SMRiQ9NPZ5. Positions 78-323.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ9NPZ5.

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni234 – 24310Interaction with galactose moiety of substrate glycoproteinBy similarity

Sequence similaritiesi

Belongs to the glycosyltransferase 43 family.Curated

Keywords - Domaini

Signal-anchor, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiNOG292627.
GeneTreeiENSGT00390000017640.
HOGENOMiHOG000261693.
HOVERGENiHBG050650.
InParanoidiQ9NPZ5.
KOiK10157.
OMAiYLTRMAN.
OrthoDBiEOG7QG44X.
PhylomeDBiQ9NPZ5.
TreeFamiTF313522.

Family and domain databases

Gene3Di3.90.550.10. 1 hit.
InterProiIPR005027. Glyco_trans_43.
IPR029044. Nucleotide-diphossugar_trans.
[Graphical view]
PANTHERiPTHR10896. PTHR10896. 1 hit.
PfamiPF03360. Glyco_transf_43. 1 hit.
[Graphical view]
SUPFAMiSSF53448. SSF53448. 1 hit.

Sequencei

Sequence statusi: Complete.

Q9NPZ5-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MKSALFTRFF ILLPWILIVI IMLDVDTRRP VPPLTPRPYF SPYAVGRGGA
60 70 80 90 100
RLPLRRGGPA HGTQKRNQSR PQPQPEPQLP TIYAITPTYS RPVQKAELTR
110 120 130 140 150
LANTFRQVAQ LHWILVEDAA ARSELVSRFL ARAGLPSTHL HVPTPRRYKR
160 170 180 190 200
PGLPRATEQR NAGLAWLRQR HQHQRAQPGV LFFADDDNTY SLELFQEMRT
210 220 230 240 250
TRKVSVWPVG LVGGRRYERP LVENGKVVGW YTGWRADRPF AIDMAGFAVS
260 270 280 290 300
LQVILSNPKA VFKRRGSQPG MQESDFLKQI TTVEELEPKA NNCTKVLVWH
310 320
TRTEKVNLAN EPKYHLDTVK IEV
Length:323
Mass (Da):36,919
Last modified:June 1, 2001 - v2
Checksum:i85058D52D2D28463
GO

Sequence cautioni

The sequence BAB70889.1 differs from that shown. Reason: Erroneous initiation. Curated
The sequence BAB85549.1 differs from that shown. Reason: Erroneous initiation. Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY070019 mRNA. Translation: AAL57718.1.
AY070110, AY070108, AY070109 Genomic DNA. Translation: AAL58977.1.
AB075843 mRNA. Translation: BAB85549.1. Different initiation.
AL121961, AL450320 Genomic DNA. Translation: CAI42145.1.
AL450320, AL121961 Genomic DNA. Translation: CAI39582.1.
AK055248 mRNA. Translation: BAB70889.1. Different initiation.
CCDSiCCDS4974.1.
RefSeqiNP_542780.1. NM_080742.2.
UniGeneiHs.713609.

Genome annotation databases

EnsembliENST00000230053; ENSP00000230053; ENSG00000112309.
GeneIDi135152.
KEGGihsa:135152.
UCSCiuc003pfv.3. human.

Cross-referencesi

Web resourcesi

GGDB

GlycoGene database

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY070019 mRNA. Translation: AAL57718.1.
AY070110, AY070108, AY070109 Genomic DNA. Translation: AAL58977.1.
AB075843 mRNA. Translation: BAB85549.1. Different initiation.
AL121961, AL450320 Genomic DNA. Translation: CAI42145.1.
AL450320, AL121961 Genomic DNA. Translation: CAI39582.1.
AK055248 mRNA. Translation: BAB70889.1. Different initiation.
CCDSiCCDS4974.1.
RefSeqiNP_542780.1. NM_080742.2.
UniGeneiHs.713609.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
2D0JX-ray2.00A/B/C/D78-323[»]
ProteinModelPortaliQ9NPZ5.
SMRiQ9NPZ5. Positions 78-323.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi9606.ENSP00000230053.

Protein family/group databases

CAZyiGT43. Glycosyltransferase Family 43.

Polymorphism and mutation databases

BioMutaiB3GAT2.
DMDMi14285363.

Proteomic databases

PaxDbiQ9NPZ5.
PRIDEiQ9NPZ5.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000230053; ENSP00000230053; ENSG00000112309.
GeneIDi135152.
KEGGihsa:135152.
UCSCiuc003pfv.3. human.

Organism-specific databases

CTDi135152.
GeneCardsiGC06M071566.
HGNCiHGNC:922. B3GAT2.
HPAiHPA012059.
MIMi607497. gene.
neXtProtiNX_Q9NPZ5.
PharmGKBiPA25216.
HUGEiSearch...
GenAtlasiSearch...

Phylogenomic databases

eggNOGiNOG292627.
GeneTreeiENSGT00390000017640.
HOGENOMiHOG000261693.
HOVERGENiHBG050650.
InParanoidiQ9NPZ5.
KOiK10157.
OMAiYLTRMAN.
OrthoDBiEOG7QG44X.
PhylomeDBiQ9NPZ5.
TreeFamiTF313522.

Enzyme and pathway databases

UniPathwayiUPA00378.
BioCyciMetaCyc:HS03557-MONOMER.
ReactomeiREACT_121408. A tetrasaccharide linker sequence is required for GAG synthesis.

Miscellaneous databases

ChiTaRSiB3GAT2. human.
EvolutionaryTraceiQ9NPZ5.
GeneWikiiB3GAT2.
GenomeRNAii135152.
NextBioi83461.
PROiQ9NPZ5.
SOURCEiSearch...

Gene expression databases

BgeeiQ9NPZ5.
CleanExiHS_B3GAT2.
ExpressionAtlasiQ9NPZ5. baseline and differential.
GenevisibleiQ9NPZ5. HS.

Family and domain databases

Gene3Di3.90.550.10. 1 hit.
InterProiIPR005027. Glyco_trans_43.
IPR029044. Nucleotide-diphossugar_trans.
[Graphical view]
PANTHERiPTHR10896. PTHR10896. 1 hit.
PfamiPF03360. Glyco_transf_43. 1 hit.
[Graphical view]
SUPFAMiSSF53448. SSF53448. 1 hit.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Cloning, characterization, and chromosome mapping of the human GlcAT-S gene."
    Marcos I., Galan J.J., Borrego S., Antinolo G.
    J. Hum. Genet. 47:677-680(2002) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
  2. "Prediction of the coding sequences of unidentified human genes. XXII. The complete sequences of 50 new cDNA clones which code for large proteins."
    Nagase T., Kikuno R., Ohara O.
    DNA Res. 8:319-327(2001) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Brain.
  3. "The DNA sequence and analysis of human chromosome 6."
    Mungall A.J., Palmer S.A., Sims S.K., Edwards C.A., Ashurst J.L., Wilming L., Jones M.C., Horton R., Hunt S.E., Scott C.E., Gilbert J.G.R., Clamp M.E., Bethel G., Milne S., Ainscough R., Almeida J.P., Ambrose K.D., Andrews T.D.
    , Ashwell R.I.S., Babbage A.K., Bagguley C.L., Bailey J., Banerjee R., Barker D.J., Barlow K.F., Bates K., Beare D.M., Beasley H., Beasley O., Bird C.P., Blakey S.E., Bray-Allen S., Brook J., Brown A.J., Brown J.Y., Burford D.C., Burrill W., Burton J., Carder C., Carter N.P., Chapman J.C., Clark S.Y., Clark G., Clee C.M., Clegg S., Cobley V., Collier R.E., Collins J.E., Colman L.K., Corby N.R., Coville G.J., Culley K.M., Dhami P., Davies J., Dunn M., Earthrowl M.E., Ellington A.E., Evans K.A., Faulkner L., Francis M.D., Frankish A., Frankland J., French L., Garner P., Garnett J., Ghori M.J., Gilby L.M., Gillson C.J., Glithero R.J., Grafham D.V., Grant M., Gribble S., Griffiths C., Griffiths M.N.D., Hall R., Halls K.S., Hammond S., Harley J.L., Hart E.A., Heath P.D., Heathcott R., Holmes S.J., Howden P.J., Howe K.L., Howell G.R., Huckle E., Humphray S.J., Humphries M.D., Hunt A.R., Johnson C.M., Joy A.A., Kay M., Keenan S.J., Kimberley A.M., King A., Laird G.K., Langford C., Lawlor S., Leongamornlert D.A., Leversha M., Lloyd C.R., Lloyd D.M., Loveland J.E., Lovell J., Martin S., Mashreghi-Mohammadi M., Maslen G.L., Matthews L., McCann O.T., McLaren S.J., McLay K., McMurray A., Moore M.J.F., Mullikin J.C., Niblett D., Nickerson T., Novik K.L., Oliver K., Overton-Larty E.K., Parker A., Patel R., Pearce A.V., Peck A.I., Phillimore B.J.C.T., Phillips S., Plumb R.W., Porter K.M., Ramsey Y., Ranby S.A., Rice C.M., Ross M.T., Searle S.M., Sehra H.K., Sheridan E., Skuce C.D., Smith S., Smith M., Spraggon L., Squares S.L., Steward C.A., Sycamore N., Tamlyn-Hall G., Tester J., Theaker A.J., Thomas D.W., Thorpe A., Tracey A., Tromans A., Tubby B., Wall M., Wallis J.M., West A.P., White S.S., Whitehead S.L., Whittaker H., Wild A., Willey D.J., Wilmer T.E., Wood J.M., Wray P.W., Wyatt J.C., Young L., Younger R.M., Bentley D.R., Coulson A., Durbin R.M., Hubbard T., Sulston J.E., Dunham I., Rogers J., Beck S.
    Nature 425:805-811(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  4. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
    Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
    , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
    Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 157-323.
    Tissue: Fetal brain.
  5. "Crystal structure of GlcAT-S, a human glucuronyltransferase, involved in the biosynthesis of the HNK-1 carbohydrate epitope."
    Shiba T., Kakuda S., Ishiguro M., Morita I., Oka S., Kawasaki T., Wakatsuki S., Kato R.
    Proteins 65:499-508(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: X-RAY CRYSTALLOGRAPHY (2.0 ANGSTROMS) OF 78-323.

Entry informationi

Entry nameiB3GA2_HUMAN
AccessioniPrimary (citable) accession number: Q9NPZ5
Secondary accession number(s): Q5JS09, Q8TF38, Q96NK4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: June 1, 2001
Last sequence update: June 1, 2001
Last modified: July 22, 2015
This is version 138 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (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.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 6
    Human chromosome 6: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  4. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  5. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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 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.