Glycosaminoglycan synthesis in fibroblasts (WP5395)

Homo sapiens

Open in new tab
Download PNG Download SVG Download JSON Download GPML Learn more about Downloads
Glycosaminoglycans (GAGs) are key components of the extracellular matrix secreted by fibroblasts. This pathway shows the metabolic reactions necessary to synthesize three types of GAGs, namely chondroitin sulfate, dermatan sulfate and heparan sulfate. The synthesis takes place in the Golgi apparatus and begins with UDP-α-D-xylose. After this molecule is converted to 3-β-D-glucosyluronate-3-β-D-galactosyl-4-β-D-galactosyl-O-β-D-xylosyl-[protein] via three metabolic reactions, the pathway separates into two branches. One of them leads to the synthesis of chondroitin sulfate and dermatan (di)sulfate, while the other ends with heparan mono-, bi- or trisulfate. Some of the reactions are catalyzed by unknown enzymes that should be researched in the future. This pathway was created based on the information from the pathways found in MetaCyc and HumanCyc databases (see Bibliography). As some of the metabolites do not have identifiers in databases supported by PathVisio (such as ChEBI or KEGG), their MetaNetX IDs were added in the comments.

Authors

Julia Błąd

Activity

last edited

Discuss this pathway

Check for ongoing discussions or start your own.

Cited In

Are you planning to include this pathway in your next publication? See How to Cite and add a link here to your paper once it's online.

Organisms

Homo sapiens

Communities

Annotations

Cell Type Ontology

fibroblast

Pathway Ontology

classic metabolic pathway

Participants
Query Drugst.One

Label Type Compact URI Comment
[heparosan]-containing α-L-iduronate Metabolite chebi:17174
3-β-D-glucosyluronate-3-β-D-galactosyl-4-β-D-galactosyl-O-β-D-xylosyl-[protein] Metabolite kegg.compound:C04903
3-β-D-galactosyl-4-β-D-galactosyl-O-β-D-xylosyl-[protein] Metabolite kegg.compound:C04825
3-O-(β-D-xylosyl)-L-seryl-[protein] Metabolite kegg.compound:C02399
dermatan-[core protein] Metabolite chebi:4439
UDP-N-acetyl-α-D-galactosamine Metabolite chebi:67138
heparosan-[core protein] Metabolite chebi:24500
deacetylated heparosan-[core protein] Metabolite kegg.compound:C02834
heparin-glucosamine-3-O-sulfate Metabolite kegg.compound:C04072
3-β-D-glucosyluronate-3-β-D-galactosyl-4-β-D-galactosyl-O-β-D-xylosyl-[protein] Metabolite chebi:80007
chondroitin 4'-sulfate Metabolite chebi:18250
[heparan sulfate] containing GlcNS Metabolite chebi:13555
4-β-D-galactosyl-O-β-D-xylosyl-[protein] Metabolite pubchem.compound:25245405
chondroitin 6'-sulfate Metabolite chebi:18296
chondroitin-[core protein] Metabolite chebi:16137
UDP-α-D-xylose Metabolite chebi:57632
UDP-α-D-galactose Metabolite chebi:66914
UDP-α-D-galactose Metabolite chebi:66914
UDP-α-D-glucuronate Metabolite chebi:58052
Dermatan-Sulfate Metabolite chebi:18376
CHST14 GeneProduct ensembl:ENSG00000169105
B3GAT2 GeneProduct ensembl:ENSG00000112309
HS2ST1 GeneProduct ensembl:ENSG00000153936
CHSY3 GeneProduct ensembl:ENSG00000198108
UST GeneProduct ensembl:ENSG00000111962
HS6ST2 GeneProduct ensembl:ENSG00000171004
CHST3 GeneProduct ensembl:ENSG00000122863
NDST4 GeneProduct ensembl:ENSG00000138653
NDST3 GeneProduct ensembl:ENSG00000164100
CHPF GeneProduct ensembl:ENSG00000123989
CHST7 GeneProduct ensembl:ENSG00000147119
CSGALNACT1 GeneProduct ensembl:ENSG00000147408
DSE GeneProduct ensembl:ENSG00000111817
B3GAT1 GeneProduct ensembl:ENSG00000109956
B4GALT7 GeneProduct ensembl:ENSG00000027847
HS3ST3A1 GeneProduct ensembl:ENSG00000153976
XYLT2 GeneProduct ensembl:ENSG00000015532
HS3ST1 GeneProduct ensembl:ENSG00000002587
GLCE GeneProduct ensembl:ENSG00000138604
CHST13 GeneProduct ensembl:ENSG00000180767
EXT2 GeneProduct ensembl:ENSG00000151348
XYLT1 GeneProduct ensembl:ENSG00000103489
HS3ST2 GeneProduct ensembl:ENSG00000122254
NDST1 GeneProduct ensembl:ENSG00000070614
HS3ST3B1 GeneProduct ensembl:ENSG00000125430
HS6ST3 GeneProduct ensembl:ENSG00000185352
EXTL2 GeneProduct ensembl:ENSG00000162694
HS6ST1 GeneProduct ensembl:ENSG00000136720
CSGALNACT2 GeneProduct ensembl:ENSG00000169826
B3GALT6 GeneProduct ensembl:ENSG00000176022
CHST12 GeneProduct ensembl:ENSG00000136213
HS3ST1 GeneProduct ensembl:ENSG00000002587
CHSY1 GeneProduct ensembl:ENSG00000131873
EXTL1 GeneProduct ensembl:ENSG00000158008
NDST2 GeneProduct ensembl:ENSG00000166507
EXTL3 GeneProduct ensembl:ENSG00000012232
CHPF2 GeneProduct ensembl:ENSG00000033100
CHST1 GeneProduct ensembl:ENSG00000175264
CHST11 GeneProduct ensembl:ENSG00000171310
B3GAT3 GeneProduct ensembl:ENSG00000149541
EXT1 GeneProduct ensembl:ENSG00000182197
CHST15 GeneProduct ensembl:ENSG00000182022
NDST4 GeneProduct ensembl:ENSG00000138653
NDST3 GeneProduct ensembl:ENSG00000164100
NDST1 GeneProduct ensembl:ENSG00000070614
NDST2 GeneProduct ensembl:ENSG00000166507
EXT2 GeneProduct ensembl:ENSG00000151348
EXT1 GeneProduct ensembl:ENSG00000182197
CSGALNACT1 GeneProduct ensembl:ENSG00000147408
CSGALNACT2 GeneProduct ensembl:ENSG00000169826
CHSY3 GeneProduct ensembl:ENSG00000198108
CHPF GeneProduct ensembl:ENSG00000123989
CHSY1 GeneProduct ensembl:ENSG00000131873

References

  1. The BioCyc collection of microbial genomes and metabolic pathways. Karp PD, Billington R, Caspi R, Fulcher CA, Latendresse M, Kothari A, et al. Brief Bioinform. 2019 Jul 19;20(4):1085–93. PubMed Europe PMC Scholia
  2. The MetaCyc database of metabolic pathways and enzymes - a 2019 update. Caspi R, Billington R, Keseler IM, Kothari A, Krummenacker M, Midford PE, et al. Nucleic Acids Res. 2020 Jan 8;48(D1):D445–53. PubMed Europe PMC Scholia
  3. Novel Insight Into Glycosaminoglycan Biosynthesis Based on Gene Expression Profiles. Huang YF, Mizumoto S, Fujita M. Front Cell Dev Biol. 2021 Sep 6;9:709018. PubMed Europe PMC Scholia
  4. Spatiotemporal diversity and regulation of glycosaminoglycans in cell homeostasis and human disease. Basu A, Patel NG, Nicholson ED, Weiss RJ. Am J Physiol Cell Physiol. 2022 May 1;322(5):C849–64. PubMed Europe PMC Scholia