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ChEBI:63516 chain [lysosomal lumen]
Stable Identifier
R-ALL-2065269
Type
Chemical Compound [SimpleEntity]
Compartment
lysosomal lumen
Synonyms
beta-D-GalNAc-(1->4)-beta-D-IdoA-(1->3)-beta-D-GalNAc4S-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl-yl group
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)
ChEBI:63516 chain [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Bos taurus)
ChEBI:63516 chain [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Bos taurus)
ChEBI:63516 chain [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)
ChEBI:63516 chain [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Caenorhabditis elegans)
ChEBI:63516 chain [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)
ChEBI:63516 chain [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Canis familiaris)
ChEBI:63516 chain [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Canis familiaris)
ChEBI:63516 chain [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)
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Danio rerio)
ChEBI:63516 chain [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)
ChEBI:63516 chain [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Dictyostelium discoideum)
ChEBI:63516 chain [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)
HEXA cleaves the terminal GalNAc from DS (Drosophila melanogaster)
ChEBI:63516 chain [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Drosophila melanogaster)
ChEBI:63516 chain [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Drosophila melanogaster)
ChEBI:63516 chain [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)
ChEBI:63516 chain [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Gallus gallus)
ChEBI:63516 chain [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Gallus gallus)
ChEBI:63516 chain [lysosomal lumen]
Disease (Homo sapiens)
Diseases of metabolism (Homo sapiens)
Diseases of glycosylation (Homo sapiens)
Diseases associated with glycosaminoglycan metabolism (Homo sapiens)
Defective HEXA causes GM2G1 (Homo sapiens)
Defective HEXA does not cleave the terminal GalNAc from DS (Homo sapiens)
ChEBI:63516 chain [lysosomal lumen]
Defective HEXB causes GM2G2 (Homo sapiens)
Defective HEXB does not cleave the terminal GalNAc from DS (Homo sapiens)
ChEBI:63516 chain [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)
ChEBI:63516 chain [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Homo sapiens)
ChEBI:63516 chain [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Homo sapiens)
ChEBI:63516 chain [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)
ChEBI:63516 chain [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Mus musculus)
ChEBI:63516 chain [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Mus musculus)
ChEBI:63516 chain [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)
ChEBI:63516 chain [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Rattus norvegicus)
ChEBI:63516 chain [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Rattus norvegicus)
ChEBI:63516 chain [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)
ChEBI:63516 chain [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Sus scrofa)
ChEBI:63516 chain [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Sus scrofa)
ChEBI:63516 chain [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)
ChEBI:63516 chain [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Xenopus tropicalis)
ChEBI:63516 chain [lysosomal lumen]
External Reference Information
External Reference
beta-D-GalNAc-(1->4)-beta-D-IdoA-(1->3)-beta-D-GalNAc4S-(1->4)-beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl-yl group [ChEBI:63516]
Participates
as an input of
Defective HEXA does not cleave the terminal GalNAc from DS (Homo sapiens)
Defective HEXB does not cleave the terminal GalNAc from DS (Homo sapiens)
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 (Drosophila melanogaster)
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 (Bos taurus)
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 (Canis familiaris)
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 (Drosophila melanogaster)
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 (Bos taurus)
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 (Canis familiaris)
as an output of
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Drosophila melanogaster)
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Gallus gallus)
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Xenopus tropicalis)
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Danio rerio)
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Sus scrofa)
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Bos taurus)
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Rattus norvegicus)
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Mus musculus)
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Homo sapiens)
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Canis familiaris)
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