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Keratan sulfate degradation
Stable Identifier
R-HSA-2022857
DOI
10.3180/R-HSA-2022857.3
Type
Pathway
Species
Homo sapiens
ReviewStatus
5/5
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Metabolism (Homo sapiens)
Metabolism of carbohydrates (Homo sapiens)
Glycosaminoglycan metabolism (Homo sapiens)
Keratan sulfate/keratin metabolism (Homo sapiens)
Keratan sulfate degradation (Homo sapiens)
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Keratan sulfate proteoglycans (KSPGs) are degraded in lysosomes as part of normal homeostasis of glycoproteins. Glycoproteins must be completely degraded to avoid undigested fragments building up and causing a variety of lysosomal storage diseases. KSPGs are Asn-linked glycoproteins and are acted upon by exo-glycosidases to release sugar monomers. The main steps of degradation are shown representing the types of cleavage reactions that occur so the full degradation of KS is not shown to avoid repetition. The proteolysis of the core protein of the glycoprotein is not shown here (Winchester 2005, Aronson & Kuranda 1989).
Literature References
PubMed ID
Title
Journal
Year
2531691
Lysosomal degradation of Asn-linked glycoproteins
Aronson NN, Jr
,
Kuranda, MJ
FASEB J
1989
15647514
Lysosomal metabolism of glycoproteins
Winchester, B
Glycobiology
2005
Participants
Events
Extracellular KSPG translocates to the lysosome for degradation
(Homo sapiens)
Keratan sulfate is cleaved from its proteoglycan by an unknown galactosidase
(Homo sapiens)
GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate
(Homo sapiens)
GLB1 hydrolyses a glycosaminoglycan
(Homo sapiens)
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate
(Homo sapiens)
HEXA cleaves the terminal GalNAc from keratan sulfate
(Homo sapiens)
HEXB cleaves the terminal GalNAc from keratan sulfate
(Homo sapiens)
Participates
as an event of
Keratan sulfate/keratin metabolism (Homo sapiens)
Event Information
Go Biological Process
keratan sulfate catabolic process (0042340)
Orthologous Events
Keratan sulfate degradation (Bos taurus)
Keratan sulfate degradation (Caenorhabditis elegans)
Keratan sulfate degradation (Canis familiaris)
Keratan sulfate degradation (Danio rerio)
Keratan sulfate degradation (Dictyostelium discoideum)
Keratan sulfate degradation (Drosophila melanogaster)
Keratan sulfate degradation (Gallus gallus)
Keratan sulfate degradation (Mus musculus)
Keratan sulfate degradation (Rattus norvegicus)
Keratan sulfate degradation (Schizosaccharomyces pombe)
Keratan sulfate degradation (Sus scrofa)
Keratan sulfate degradation (Xenopus tropicalis)
Authored
Jassal, B (2011-12-01)
Reviewed
D'Eustachio, P (2012-03-28)
Created
Jassal, B (2011-12-01)
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