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GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Stable Identifier
R-ALL-2105012
Type
Chemical Compound [SimpleEntity]
Compartment
lysosomal lumen
Synonyms
beta-D-GlcpNAc-(1->3)-beta-D-Galp-(1->4)-beta-D-GlcpNAc6S-(1->3)-D-Galp
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 (Bos taurus)
Glycosaminoglycan metabolism (Bos taurus)
Keratan sulfate/keratin metabolism (Bos taurus)
Keratan sulfate degradation (Bos taurus)
HEXA cleaves the terminal GalNAc from keratan sulfate (Bos taurus)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Bos taurus)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Bos taurus)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Metabolism (Caenorhabditis elegans)
Metabolism of carbohydrates (Caenorhabditis elegans)
Glycosaminoglycan metabolism (Caenorhabditis elegans)
Keratan sulfate/keratin metabolism (Caenorhabditis elegans)
Keratan sulfate degradation (Caenorhabditis elegans)
HEXA cleaves the terminal GalNAc from keratan sulfate (Caenorhabditis elegans)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Caenorhabditis elegans)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Metabolism (Canis familiaris)
Metabolism of carbohydrates (Canis familiaris)
Glycosaminoglycan metabolism (Canis familiaris)
Keratan sulfate/keratin metabolism (Canis familiaris)
Keratan sulfate degradation (Canis familiaris)
HEXA cleaves the terminal GalNAc from keratan sulfate (Canis familiaris)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Canis familiaris)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Canis familiaris)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Metabolism (Danio rerio)
Metabolism of carbohydrates (Danio rerio)
Glycosaminoglycan metabolism (Danio rerio)
Keratan sulfate/keratin metabolism (Danio rerio)
Keratan sulfate degradation (Danio rerio)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Danio rerio)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Metabolism (Dictyostelium discoideum)
Metabolism of carbohydrates (Dictyostelium discoideum)
Glycosaminoglycan metabolism (Dictyostelium discoideum)
Keratan sulfate/keratin metabolism (Dictyostelium discoideum)
Keratan sulfate degradation (Dictyostelium discoideum)
HEXA cleaves the terminal GalNAc from keratan sulfate (Dictyostelium discoideum)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Dictyostelium discoideum)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Metabolism (Drosophila melanogaster)
Metabolism of carbohydrates (Drosophila melanogaster)
Glycosaminoglycan metabolism (Drosophila melanogaster)
Keratan sulfate/keratin metabolism (Drosophila melanogaster)
Keratan sulfate degradation (Drosophila melanogaster)
HEXA cleaves the terminal GalNAc from keratan sulfate (Drosophila melanogaster)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Drosophila melanogaster)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Drosophila melanogaster)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Metabolism (Gallus gallus)
Metabolism of carbohydrates (Gallus gallus)
Glycosaminoglycan metabolism (Gallus gallus)
Keratan sulfate/keratin metabolism (Gallus gallus)
Keratan sulfate degradation (Gallus gallus)
HEXA cleaves the terminal GalNAc from keratan sulfate (Gallus gallus)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Gallus gallus)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Gallus gallus)
GlcNAc-Gal-GlcNAc(S)-Gal [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 keratan sulfate (Homo sapiens)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Defective HEXB causes GM2G2 (Homo sapiens)
Defective HEXB does not cleave the terminal GalNAc from keratan sulfate (Homo sapiens)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Metabolism (Homo sapiens)
Metabolism of carbohydrates (Homo sapiens)
Glycosaminoglycan metabolism (Homo sapiens)
Keratan sulfate/keratin metabolism (Homo sapiens)
Keratan sulfate degradation (Homo sapiens)
HEXA cleaves the terminal GalNAc from keratan sulfate (Homo sapiens)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Homo sapiens)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Homo sapiens)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Metabolism (Mus musculus)
Metabolism of carbohydrates (Mus musculus)
Glycosaminoglycan metabolism (Mus musculus)
Keratan sulfate/keratin metabolism (Mus musculus)
Keratan sulfate degradation (Mus musculus)
HEXA cleaves the terminal GalNAc from keratan sulfate (Mus musculus)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Mus musculus)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Mus musculus)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Metabolism (Rattus norvegicus)
Metabolism of carbohydrates (Rattus norvegicus)
Glycosaminoglycan metabolism (Rattus norvegicus)
Keratan sulfate/keratin metabolism (Rattus norvegicus)
Keratan sulfate degradation (Rattus norvegicus)
HEXA cleaves the terminal GalNAc from keratan sulfate (Rattus norvegicus)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Rattus norvegicus)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Rattus norvegicus)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Metabolism (Sus scrofa)
Metabolism of carbohydrates (Sus scrofa)
Glycosaminoglycan metabolism (Sus scrofa)
Keratan sulfate/keratin metabolism (Sus scrofa)
Keratan sulfate degradation (Sus scrofa)
HEXA cleaves the terminal GalNAc from keratan sulfate (Sus scrofa)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Sus scrofa)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Sus scrofa)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
Metabolism (Xenopus tropicalis)
Metabolism of carbohydrates (Xenopus tropicalis)
Glycosaminoglycan metabolism (Xenopus tropicalis)
Keratan sulfate/keratin metabolism (Xenopus tropicalis)
Keratan sulfate degradation (Xenopus tropicalis)
HEXB cleaves the terminal GalNAc from keratan sulfate (Xenopus tropicalis)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Xenopus tropicalis)
GlcNAc-Gal-GlcNAc(S)-Gal [lysosomal lumen]
External Reference Information
External Reference
beta-D-GlcpNAc-(1->3)-beta-D-Galp-(1->4)-beta-D-GlcpNAc6S-(1->3)-D-Galp [ChEBI:63852]
Participates
as an input of
Defective HEXA does not cleave the terminal GalNAc from keratan sulfate (Homo sapiens)
Defective HEXB does not cleave the terminal GalNAc from keratan sulfate (Homo sapiens)
HEXB cleaves the terminal GalNAc from keratan sulfate (Dictyostelium discoideum)
HEXB cleaves the terminal GalNAc from keratan sulfate (Caenorhabditis elegans)
HEXB cleaves the terminal GalNAc from keratan sulfate (Drosophila melanogaster)
HEXB cleaves the terminal GalNAc from keratan sulfate (Gallus gallus)
HEXB cleaves the terminal GalNAc from keratan sulfate (Xenopus tropicalis)
HEXB cleaves the terminal GalNAc from keratan sulfate (Sus scrofa)
HEXB cleaves the terminal GalNAc from keratan sulfate (Bos taurus)
HEXB cleaves the terminal GalNAc from keratan sulfate (Canis familiaris)
HEXB cleaves the terminal GalNAc from keratan sulfate (Rattus norvegicus)
HEXB cleaves the terminal GalNAc from keratan sulfate (Mus musculus)
HEXB cleaves the terminal GalNAc from keratan sulfate (Homo sapiens)
HEXA cleaves the terminal GalNAc from keratan sulfate (Dictyostelium discoideum)
HEXA cleaves the terminal GalNAc from keratan sulfate (Caenorhabditis elegans)
HEXA cleaves the terminal GalNAc from keratan sulfate (Drosophila melanogaster)
HEXA cleaves the terminal GalNAc from keratan sulfate (Gallus gallus)
HEXA cleaves the terminal GalNAc from keratan sulfate (Sus scrofa)
HEXA cleaves the terminal GalNAc from keratan sulfate (Bos taurus)
HEXA cleaves the terminal GalNAc from keratan sulfate (Canis familiaris)
HEXA cleaves the terminal GalNAc from keratan sulfate (Rattus norvegicus)
HEXA cleaves the terminal GalNAc from keratan sulfate (Mus musculus)
HEXA cleaves the terminal GalNAc from keratan sulfate (Homo sapiens)
as an output of
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Drosophila melanogaster)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Gallus gallus)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Xenopus tropicalis)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Danio rerio)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Sus scrofa)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Bos taurus)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Canis familiaris)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Rattus norvegicus)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Mus musculus)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Homo sapiens)
Cross References
KEGG Glycan
G13033
ZINC
ZINC000096085300
,
ZINC000096085299
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