UniProt:Q93063 EXT2

chain
  • chain:1-718
checksum 9048CD3A5B63C5CB
comment
  • FUNCTION Glycosyltransferase forming with EXT1 the heterodimeric heparan sulfate polymerase which catalyzes the elongation of the heparan sulfate glycan backbone (PubMed:22660413, PubMed:36402845, PubMed:36593275). Glycan backbone extension consists in the alternating transfer of (1->4)-beta-D-GlcA and (1->4)-alpha-D-GlcNAc residues from their respective UDP-sugar donors. Both EXT1 and EXT2 are required for the full activity of the polymerase since EXT1 bears the N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity within the complex while EXT2 carries the glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity (PubMed:36402845, PubMed:36593275). Heparan sulfate proteoglycans are ubiquitous components of the extracellular matrix and play an important role in tissue homeostasis and signaling (PubMed:19344451, PubMed:22660413).CATALYTIC ACTIVITY 3-O-{[(1->4)-beta-D-GlcA-(1->4)-alpha-D-GlcNAc](n)-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl-[protein] + UDP-N-acetyl-alpha-D-glucosamine = 3-O-{alpha-D-GlcNAc-[(1->4)-beta-D-GlcA-(1->4)-alpha-D-GlcNAc](n)-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl}-L-seryl-[protein] + UDP + H(+)COFACTOR Protein modification; protein glycosylation.SUBUNIT Part of the heparan sulfate polymerase, a dimeric complex composed of EXT1 and EXT2 (PubMed:10679296, PubMed:36402845, PubMed:36593275). Could also form homooligomeric complexes (PubMed:10679296). Interacts with NDST1 (PubMed:18337501, PubMed:35137078). Interacts with GALNT5 (PubMed:10545594).INTERACTION The active heparan sulfate polymerase complex composed of EXT1 and EXT2 is localized to the Golgi apparatus. If both proteins are individually detected in the endoplasmic reticulum, the formation of the complex promotes their transport to the Golgi.ALTERNATIVE PRODUCTS Widely expressed.PTM N-glycosylated at Asn-637.PTM A soluble form is generated by proteolytic processing.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the glycosyltransferase 47 family.
crossReference
databaseName UniProt
dbId 54544
description
  • recommendedName: fullName evidence="25"Exostosin-2 ecNumber evidence="19"2.4.1.224 alternativeName: fullName evidence="27"Exostosin glycosyltransferase 2 alternativeName: fullName evidence="23"Glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase alternativeName: fullName evidence="23"Heparan sulfate co-polymerase subunit EXT1 alternativeName: fullName evidence="24"Multiple exostoses protein 2
displayName UniProt:Q93063 EXT2
geneName
  • EXT2
identifier Q93063
isSequenceChanged false
keyword
  • 3D-structure
  • Alternative splicing
  • Disease variant
  • Disulfide bond
  • Endoplasmic reticulum
  • Epilepsy
  • Glycoprotein
  • Glycosyltransferase
  • Golgi apparatus
  • Hereditary multiple exostoses
  • Intellectual disability
  • Manganese
  • Membrane
  • Metal-binding
  • Proteomics identification
  • Reference proteome
  • Secreted
  • Signal-anchor
  • Transferase
  • Transmembrane
  • Transmembrane helix
  • Tumor suppressor
modified [InstanceEdit:9939033] Weiser, Joel, 2025-02-21
moleculeType Protein
name
  • EXT2
otherIdentifier
  • 11723014_a_at
  • 11723015_s_at
  • 11752434_a_at
  • 11752435_s_at
  • 16724031
  • 202012_PM_s_at
  • 202012_s_at
  • 202013_PM_s_at
  • 202013_s_at
  • 2132
  • 235685_PM_at
  • 235685_at
  • 236483_PM_at
  • 236483_at
  • 3328390
  • 3328391
  • 3328392
  • 3328393
  • 3328394
  • 3328395
  • 3328396
  • 3328397
  • 3328400
  • 3328401
  • 3328402
  • 3328403
  • 3328404
  • 3328405
  • 3328409
  • 3328410
  • 3328411
  • 3328415
  • 3328416
  • 3328417
  • 3328418
  • 3328420
  • 3328426
  • 3328427
  • 3328428
  • 3328429
  • 3328430
  • 3328431
  • 3328432
  • 3328433
  • 3328434
  • 3328442
  • 3328454
  • 3328455
  • 3328456
  • 3328457
  • 3328458
  • 3328460
  • 3328462
  • 36593_at
  • 36594_s_at
  • 54876_at
  • 57976_at
  • 7939524
  • A_14_P102151
  • A_14_P103622
  • A_14_P104744
  • A_14_P110150
  • A_23_P13183
  • A_32_P27373
  • GE58218
  • GE611937
  • GE854284
  • GE870432
  • GO:0000139
  • GO:0000271
  • GO:0001503
  • GO:0001707
  • GO:0002062
  • GO:0003013
  • GO:0005515
  • GO:0005576
  • GO:0005783
  • GO:0005789
  • GO:0005794
  • GO:0005975
  • GO:0006024
  • GO:0006486
  • GO:0006487
  • GO:0006790
  • GO:0008217
  • GO:0008375
  • GO:0010467
  • GO:0015012
  • GO:0015020
  • GO:0016020
  • GO:0016740
  • GO:0016757
  • GO:0030154
  • GO:0030210
  • GO:0042044
  • GO:0042311
  • GO:0042328
  • GO:0043541
  • GO:0044344
  • GO:0046872
  • GO:0046982
  • GO:0050508
  • GO:0050509
  • GO:0050891
  • GO:0051923
  • GO:0055078
  • GO:0060047
  • GO:0060350
  • GO:0070062
  • GO:1901135
  • GO:1902494
  • HMNXSV003043488
  • Hs.100265.0.A1_3p_at
  • Hs.103682.0.A1_3p_at
  • Hs.75334.0.S1_3p_a_at
  • ILMN_1761259
  • ILMN_2328029
  • PH_hs_0004312
  • TC11003446.hg
  • U67368_s_at
  • U72263_s_at
  • g4557572_3p_a_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • EXT2_HUMAN
  • B2R5Z6
  • C9JU51
  • J3KPT2
  • O15288
sequenceLength 718
species [Species:48887] Homo sapiens
stId uniprot:Q93063
url http://purl.uniprot.org/uniprot/Q93063
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