Glycosaminoglycan synthesis in fibroblasts (WP5395)
Homo sapiens
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 and Eric WeitzActivity
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Organisms
Homo sapiensCommunities
Annotations
Cell Type Ontology
fibroblastPathway Ontology
classic metabolic pathway glycosaminoglycan metabolic pathway| 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-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 | |
| 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 |
References
- 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
- 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
- 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
- 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