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IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Stable Identifier
R-ALL-2105025
Type
Chemical Compound [SimpleEntity]
Compartment
lysosomal lumen
Synonyms
beta-D-IdopA-(1->3)-beta-D-GalpNAc4S-(1->4)-beta-D-GlcpA-(1->3)-beta-D-Galp-(1->3)-beta-D-Galp-(1->4)-beta-D-Xylp
Locations in the PathwayBrowser
for Species:
Homo sapiens
Bos taurus
Caenorhabditis elegans
Canis familiaris
Danio rerio
Dictyostelium discoideum
Drosophila melanogaster
Gallus gallus
Mus musculus
Rattus norvegicus
Sus scrofa
Xenopus tropicalis
Expand all
Metabolism (Bos taurus)
Metabolism of carbohydrates and carbohydrate derivatives (Bos taurus)
Glycosaminoglycan metabolism (Bos taurus)
Chondroitin sulfate/dermatan sulfate metabolism (Bos taurus)
CS/DS degradation (Bos taurus)
HEXA cleaves the terminal GalNAc from DS (Bos taurus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Bos taurus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Bos taurus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Metabolism (Caenorhabditis elegans)
Metabolism of carbohydrates and carbohydrate derivatives (Caenorhabditis elegans)
Glycosaminoglycan metabolism (Caenorhabditis elegans)
Chondroitin sulfate/dermatan sulfate metabolism (Caenorhabditis elegans)
CS/DS degradation (Caenorhabditis elegans)
HEXA cleaves the terminal GalNAc from DS (Caenorhabditis elegans)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Caenorhabditis elegans)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Metabolism (Canis familiaris)
Metabolism of carbohydrates and carbohydrate derivatives (Canis familiaris)
Glycosaminoglycan metabolism (Canis familiaris)
Chondroitin sulfate/dermatan sulfate metabolism (Canis familiaris)
CS/DS degradation (Canis familiaris)
HEXA cleaves the terminal GalNAc from DS (Canis familiaris)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Canis familiaris)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Canis familiaris)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Metabolism (Danio rerio)
Metabolism of carbohydrates and carbohydrate derivatives (Danio rerio)
Glycosaminoglycan metabolism (Danio rerio)
Chondroitin sulfate/dermatan sulfate metabolism (Danio rerio)
CS/DS degradation (Danio rerio)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Danio rerio)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Metabolism (Dictyostelium discoideum)
Metabolism of carbohydrates and carbohydrate derivatives (Dictyostelium discoideum)
Glycosaminoglycan metabolism (Dictyostelium discoideum)
Chondroitin sulfate/dermatan sulfate metabolism (Dictyostelium discoideum)
CS/DS degradation (Dictyostelium discoideum)
HEXA cleaves the terminal GalNAc from DS (Dictyostelium discoideum)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Dictyostelium discoideum)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Dictyostelium discoideum)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Metabolism (Drosophila melanogaster)
Metabolism of carbohydrates and carbohydrate derivatives (Drosophila melanogaster)
Glycosaminoglycan metabolism (Drosophila melanogaster)
Chondroitin sulfate/dermatan sulfate metabolism (Drosophila melanogaster)
CS/DS degradation (Drosophila melanogaster)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Drosophila melanogaster)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Metabolism (Gallus gallus)
Metabolism of carbohydrates and carbohydrate derivatives (Gallus gallus)
Glycosaminoglycan metabolism (Gallus gallus)
Chondroitin sulfate/dermatan sulfate metabolism (Gallus gallus)
CS/DS degradation (Gallus gallus)
HEXA cleaves the terminal GalNAc from DS (Gallus gallus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Gallus gallus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Gallus gallus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Disease (Homo sapiens)
Diseases of metabolism (Homo sapiens)
Diseases of carbohydrate metabolism (Homo sapiens)
Mucopolysaccharidoses (Homo sapiens)
MPS I - Hurler syndrome (CS/DS degradation) (Homo sapiens)
Defective IDUA does not hydrolyse the unsulfated alpha-L-iduronosidic link in DS (Homo sapiens)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Metabolism (Homo sapiens)
Metabolism of carbohydrates and carbohydrate derivatives (Homo sapiens)
Glycosaminoglycan metabolism (Homo sapiens)
Chondroitin sulfate/dermatan sulfate metabolism (Homo sapiens)
CS/DS degradation (Homo sapiens)
HEXA cleaves the terminal GalNAc from DS (Homo sapiens)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Homo sapiens)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Homo sapiens)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Metabolism (Mus musculus)
Metabolism of carbohydrates and carbohydrate derivatives (Mus musculus)
Glycosaminoglycan metabolism (Mus musculus)
Chondroitin sulfate/dermatan sulfate metabolism (Mus musculus)
CS/DS degradation (Mus musculus)
HEXA cleaves the terminal GalNAc from DS (Mus musculus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Mus musculus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Mus musculus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Metabolism (Rattus norvegicus)
Metabolism of carbohydrates and carbohydrate derivatives (Rattus norvegicus)
Glycosaminoglycan metabolism (Rattus norvegicus)
Chondroitin sulfate/dermatan sulfate metabolism (Rattus norvegicus)
CS/DS degradation (Rattus norvegicus)
HEXA cleaves the terminal GalNAc from DS (Rattus norvegicus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Rattus norvegicus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Rattus norvegicus)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Metabolism (Sus scrofa)
Metabolism of carbohydrates and carbohydrate derivatives (Sus scrofa)
Glycosaminoglycan metabolism (Sus scrofa)
Chondroitin sulfate/dermatan sulfate metabolism (Sus scrofa)
CS/DS degradation (Sus scrofa)
HEXA cleaves the terminal GalNAc from DS (Sus scrofa)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Sus scrofa)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Sus scrofa)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
Metabolism (Xenopus tropicalis)
Metabolism of carbohydrates and carbohydrate derivatives (Xenopus tropicalis)
Glycosaminoglycan metabolism (Xenopus tropicalis)
Chondroitin sulfate/dermatan sulfate metabolism (Xenopus tropicalis)
CS/DS degradation (Xenopus tropicalis)
HEXB cleaves the terminal GalNAc from DS (Xenopus tropicalis)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Xenopus tropicalis)
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl [lysosomal lumen]
External Reference Information
External Reference
beta-D-IdopA-(1->3)-beta-D-GalpNAc4S-(1->4)-beta-D-GlcpA-(1->3)-beta-D-Galp-(1->3)-beta-D-Galp-(1->4)-beta-D-Xylp [ChEBI:63873]
Participates
as an input of
Defective IDUA does not hydrolyse the unsulfated alpha-L-iduronosidic link in DS (Homo sapiens)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Dictyostelium discoideum)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Drosophila melanogaster)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Gallus gallus)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Xenopus tropicalis)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Danio rerio)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Sus scrofa)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Canis familiaris)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Rattus norvegicus)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Mus musculus)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Homo sapiens)
IDUA hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Bos taurus)
as an output of
HEXB cleaves the terminal GalNAc from DS (Dictyostelium discoideum)
HEXB cleaves the terminal GalNAc from DS (Caenorhabditis elegans)
HEXB cleaves the terminal GalNAc from DS (Gallus gallus)
HEXB cleaves the terminal GalNAc from DS (Xenopus tropicalis)
HEXB cleaves the terminal GalNAc from DS (Sus scrofa)
HEXB cleaves the terminal GalNAc from DS (Canis familiaris)
HEXB cleaves the terminal GalNAc from DS (Rattus norvegicus)
HEXB cleaves the terminal GalNAc from DS (Mus musculus)
HEXB cleaves the terminal GalNAc from DS (Homo sapiens)
HEXB cleaves the terminal GalNAc from DS (Bos taurus)
HEXA cleaves the terminal GalNAc from DS (Dictyostelium discoideum)
HEXA cleaves the terminal GalNAc from DS (Caenorhabditis elegans)
HEXA cleaves the terminal GalNAc from DS (Gallus gallus)
HEXA cleaves the terminal GalNAc from DS (Sus scrofa)
HEXA cleaves the terminal GalNAc from DS (Canis familiaris)
HEXA cleaves the terminal GalNAc from DS (Rattus norvegicus)
HEXA cleaves the terminal GalNAc from DS (Mus musculus)
HEXA cleaves the terminal GalNAc from DS (Homo sapiens)
HEXA cleaves the terminal GalNAc from DS (Bos taurus)
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