5.1.3.19: chondroitin-glucuronate 5-epimerase
This is an abbreviated version!
For detailed information about chondroitin-glucuronate 5-epimerase, go to the full flat file.
Word Map on EC 5.1.3.19
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5.1.3.19
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iduronic
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glycosaminoglycans
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proteoglycans
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epimerization
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decorin
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d-glucuronic
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ehlers-danlos
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musculocontractural
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chst14
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4-o-sulfotransferase
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mceds
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idoa
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4-o-sulfated
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analysis
- 5.1.3.19
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iduronic
- glycosaminoglycans
- proteoglycans
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epimerization
- decorin
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d-glucuronic
- ehlers-danlos
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musculocontractural
- chst14
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4-o-sulfotransferase
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mceds
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idoa
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4-o-sulfated
- analysis
Reaction
Synonyms
Chondroitin D-glucuronosyl 5-epimerase, chondroitin-glucuronate C5-epimerase, dermatan sulfate epimerase, dermatan sulfate epimerase 1, dermatan sulfate epimerase 2, dermatan sulfate epimerase like, dermatan sulfate epimerase-2, dermatan sulfate epimerase-like a, dermatan sulfate epimerase-like b, Dermatan-sulfate 5-epimerase, DS epimerase 1, DS epimerase 2, DS-epi1, DS-epi2, DS-epimerase 1, DSE, DSEL, dsela, dselb, Epimerase, chondroitin glucuronate 5-, Polyglucuronate 5-epimerase, SART2
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5.1.3.19 chondroitin-glucuronate 5-epimeraseAdvanced search results
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General Information on EC 5.1.3.19 - chondroitin-glucuronate 5-epimerase
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GENERAL INFORMATION 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
evolution
conversion of GlcA residues into the stereoisomer IdoA is mediated by two dermatan sulfate epimerases, DSE (DS-epi1) and DSEL (DS-epi2)
malfunction
metabolism
physiological function
additional information
malfunction
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DS-epi1 deficiency alters skin morphology, collagen fibril ultrastructure, and skin tensile strength, phenotype, overview
malfunction
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DS-epi2 is genetically linked to bipolar disorder, which suggests that the dermatan sulfate domains generated by a defective enzyme may be involved in the etiology of the disease
malfunction
a naturally occuring homozygous DSE mutation c.803C>T, pS268L, causes musculocontractural type of Ehlers-Danlos syndrome, MCEDS. The mutant enzymes shows a loss of activity towards partially desulfated dermatan sulfate, patient-derived fibroblasts also show a significant reduction in epimerase activity. Dermatan sulfate disaccharides are reduced, while chondroitin sulfate disaccharides are increased in cultured fibroblasts. Stable transfection of patient fibroblasts with a DSE expression vector increases the amount of secreted dermatan sulfate disaccharides
metabolism
the enzyme catalyzes a step in dermatan sulfate and chondroitin sulfate biosynthesis, pathway overview
metabolism
the enzyme catalyzes a step in dermatan sulfate and chondroitin sulfte biosynthesis, pathway overview
metabolism
the enzyme is involved in the chondroitin/dermatan sulfate biosynthesis, overview
physiological function
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DS-epi2 and DS-epi1 are both involved in the biosynthesis of the iduronic acid blocks in fibroblasts and that DS-epi2 can also synthesize the hybrid structures
physiological function
isoform DS-epi2 plays key roles in the epimerization of chondroitin sulfate and dermatan sulfate during its biosynthesis. The L-iduronate-containing structures in the developing brain are mainly produced by the actions of isoform DS-epi2 and play crucial roles in postnatal development
physiological function
the enzyme is important in human development and extracellular matrix maintenance
physiological function
conversion of GlcA residues into the stereoisomer IdoA is mediated by two dermatan sulfate epimerases, DSE (DS-epi1) and DSEL (DS-epi2), modification reactions in chondroitin/dermatan sulfate biosynthesis, overview
physiological function
the enzyme converts chondroitin sulfate (CS) to a CS/dermatan sulfate (DS) hybrid chain, which is structurally and conformationally richer than CS, favouring interaction with matrix proteins and growth factors
physiological function
the enzyme converts chondroitin sulfate (CS) to a CS/dermatan sulfate (DS) hybrid chain, which is structurally and conformationally richer than CS, favouring interaction with matrix proteins and growth factors. Xenopus Dse is essential for the migration of neural crest cells by allowing cell surface CS/DS proteoglycans to adhere to fibronectin
additional information
enzyme Dsel harbors a C-terminal sulfotransferase-like domain
additional information
enzyme Dsel harbors a C-terminal sulfotransferase-like domain
additional information
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polysaccharide processivity opens up the possibility for an efficient formation of long stretches of IdoA, which have been shown to be of major importance for CS/DS regulatory effect on collagen fibrillization
