Glycosaminoglycan-protein linkage region biosynthesis

Summary
Organism
Homo sapiens (human)
Reactome
R-HSA-1971475
PubChem
R-HSA-1971475
Description
  • The biosynthesis of dermatan sulfate/chondroitin sulfate and heparin/heparan sulfate glycosaminoglycans (GAGs) starts with the formation of a tetrasaccharide linker sequence to the core protein. The first step is the addition of xylose to the hydroxy group of specific serine residues on the core protein. Subsequent additions of two galactoses and a glucuronide moiety completes the linker sequence. From here, the next hexosamine addition is critical as it determines which GAG is formed (Lamberg & Stoolmiller 1974, Pavao et al. 2006).
Click on a node on the pathway to see its details. Glycoproteins are marked with a glycoprotein icon in their name.
Displaying entries 1 - 10 of 29 in total
UniProt ID Protein Name Gene Symbol Pathway Viewer
O00468 Agrin
  • AGRIN
  • AGRN
view
O14594 Neurocan core protein
  • CSPG3
  • NCAN
  • NEUR
view
O75056 Syndecan-3
  • KIAA0468
  • SDC3
view
O75063 Glycosaminoglycan xylosylkinase
  • FAM20B
  • KIAA0475
view
O75487 Glypican-4
  • GPC4
  • UNQ474/PRO937
view
O94766 Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3
  • B3GAT3
  • hCG_16017
view
O95196 Chondroitin sulfate proteoglycan 5
  • CALEB
  • CSPG5
  • NGC
view
P07585 Decorin
  • DCN
  • DKFZp686J19238
  • SLRR1B
  • hCG_24110
view
P13611 Versican core protein
  • CSPG2
  • VCAN
view
P18827 Syndecan-1
  • SDC
  • SDC1
view
Displaying all 5 entries
GlyCosmos Lectin UniProt ID Lectin Name Pathway Viewer
GL_000109 Q96GW7 Brevican core protein view
GL_000807 O14594 Neurocan core protein view
GL_001169 P13611 Versican core protein view
GL_005327 O00468 Agrin view
GL_005334 P98160 Basement membrane-specific heparan sulfate proteoglycan core protein view

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Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01


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Last updated: April 6, 2026