| Entry |
|
| Name |
Glycosaminoglycan biosynthesis - keratan sulfate
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| Description |
Keratan sulfate (KS) is a glycosaminoglycan with the basic disaccharide unit of N-acetyllactosamine, Gal(b1-4)GlcNAc(b1-3), with sulfate esters at C-6 of GlcNAc and Gal residues. There are two types of KS distinguished by the protein linkage: type I for N-linked via the N-glycan core structure and type II for O-linked via the O-glycan core 2 structure.
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| Class |
Metabolism; Glycan biosynthesis and metabolism
 |
| Pathway map |
| Glycosaminoglycan biosynthesis - keratan sulfate |

  |
| Disease |
| Nonsyndromic autosomal recessive mental retardation (NS-ARMR) | | Macular corneal dystrophy (MCD) |
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| Orthology |
| glycoprotein 6-alpha-L-fucosyltransferase [EC: 2.4.1.68] | | | | | | | | N-acetyllactosaminide beta-1,3-N-acetylglucosaminyltransferase [EC: 2.4.1.149] | | carbohydrate 6-sulfotransferase 6 [EC:2.8.2.-] | | beta-1,4-galactosyltransferase 4 [EC:2.4.1.- 2.4.1.90] | | beta-1,3-N-acetylglucosaminyltransferase 7 [EC:2.4.1.-] | | keratan sulfate 6-sulfotransferase 1 [EC: 2.8.2.21] | | N-acetyllactosaminide alpha-2,3-sialyltransferase (sialyltransferase 6) [EC: 2.4.99.6] | | carbohydrate 6-sulfotransferase 2 [EC:2.8.2.-] | | carbohydrate 6-sulfotransferase 4 [EC:2.8.2.-] | | beta-galactoside alpha-2,3-sialyltransferase (sialyltransferase 4A) [EC: 2.4.99.4] | | beta-galactoside alpha-2,3-sialyltransferase (sialyltransferase 4B) [EC: 2.4.99.4] |
|
| Reference |
|
| Authors |
Funderburgh JL. |
| Title |
Keratan sulfate: structure, biosynthesis, and function. |
| Journal |
Glycobiology 10:951-958 (2000) |
| Reference |
|
| Authors |
Seko A, Yamashita K. |
| Title |
beta1,3-N-Acetylglucosaminyltransferase-7 (beta3Gn-T7) acts efficiently on keratan sulfate-related glycans. |
| Journal |
FEBS Lett 556:216-20 (2004) |
| Reference |
|
| Authors |
Seko A, Dohmae N, Takio K, Yamashita K. |
| Title |
Beta 1,4-galactosyltransferase (beta 4GalT)-IV is specific for GlcNAc 6-O-sulfate. Beta 4GalT-IV acts on keratan sulfate-related glycans and a precursor glycan of 6-sulfosialyl-Lewis X. |
| Journal |
J Biol Chem 278:9150-8 (2003) |