Pathway
Keratan sulfate/keratin metabolism
Keratan sulfate (KS) (a glycosaminoglycan, GAG) is a linear polysaccharide that consists of the repeating disaccharide unit GlcNAc-Gal (N-acetylglucosamine-galactose). KS can perform a structural function and is found in bone, cartilage and the cornea. In joints, it also acts as a shock absorber due to its highly hydrated nature. There are several classes of KS, KSI, II and III. KSI is N-linked to asparagine (Asn) residues in the core protein and is predominantly found in the cornea. KSII is O-linked to serine (Ser) or Thr (threonine) residues in the core protein and is found predominantly in cartilage linked to the protein aggrecan, forming the most abundant proteoglycan in cartilage. A third class of KS, KSIII, are proteoglycans in the brain. KSIII chains are linked to Ser/Thr residues in the core protein via mannose.
Continue reading
Normally, the body degrades GAGs as a natural turnover. Defects in the degradative enzymes cause the autosomal recessive mucopolysaccharide storage disease Morquio's syndrome (also called mucopolysaccharidosis IV). This involves the build up of KS in lysosomes, manifesting clinically as skeletal, dental and corneal abnormalities.
What the reference databases state
Statements
36 statements
36 statements · 1 reference database · Subject: Keratan sulfate/keratin metabolism · Sources: Reactome
- ACAN participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- B3GNT2 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- B3GNT3 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- B3GNT4 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- B3GNT7 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- B4GALT1 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- B4GALT2 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- B4GALT3 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- B4GALT4 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- B4GALT5 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- B4GALT6 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- B4GAT1 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
Show the remaining 24
- CHST1 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- CHST2 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- CHST3 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- CHST5 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- CHST6 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- FMOD participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- GALNS participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- GLB1 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- GLB1L participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- GLB1L2 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- GLB1L3 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- GNS participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- HEXA participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- HEXB participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- LUM participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- OGN participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- OMD participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- PRELP participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- SLC35D2 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- ST3GAL1 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- ST3GAL2 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- ST3GAL3 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- ST3GAL4 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only
- ST3GAL6 participates in Keratan sulfate/keratin metabolismProteinReactome R-HSA-1638074Inferred only