Keratan sulfate/keratin metabolism

Tomatsu S, Montaño AM, Nishioka T, Gutierrez MA, Peña OM, Tranda Firescu GG, Lopez P, Yamaguchi S, Noguchi A, Orii T. Mutation and polymorphism spectrum of the GALNS gene in mucopolysaccharidosis IVA (Morquio A). Hum Mutat. 2005 Dec;26(6):500–12. doi: 10.1002/humu.20257. PMID: 16287098.; Funderburgh JL. Keratan sulfate biosynthesis. IUBMB Life. 2002 Oct;54(4):187–94. PMID: 12512857; PMCID: PMC2874674.; Funderburgh JL. Keratan sulfate: structure, biosynthesis, and function. Glycobiology. 2000 Oct;10(10):951–8. doi: 10.1093/glycob/10.10.951. PMID: 11030741.

Metabolites

alpha-D-Galactose

Formula: C6H12O6 (180.0633852)

CAS ID: 3646-73-9

Calcium cation

Formula: Ca (39.962591)

CAS ID: 14127-61-8

H+

Formula: H (1.0078246)

CAS ID: 12408-02-5

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Manganese(2+)

Formula: Mn (54.938046)

CAS ID: 7439-96-5

Succinate

Formula: C4H6O4 (118.0266076)

CAS ID: 110-15-6

Sulfate

Formula: H2SO4 (97.9673812)

CAS ID: 7664-93-9

UDP(3-)

Formula: C9H11N2O12P2 (400.9787246)

CAS ID: 86527-70-0



Enzyme

EC Number name full name note
2.4.1.-
2.4.1.22 lactose synthase UDP-galactose:D-glucose 4-beta-D-galactosyltransferase
2.4.1.274 glucosylceramide beta-1,4-galactosyltransferase UDP-galactose:beta-D-glucosyl-(1<->1)-ceramide beta-1,4-galactosyltransferase
2.4.1.275 neolactotriaosylceramide beta-1,4-galactosyltransferase UDP-alpha-D-galactose:N-acetyl-beta-D-glucosaminyl-(1->3)-beta-D-galactosyl-(1beta4)-beta-D-glucosyl-(1<->1)-ceramide 4-beta-D-galactosyltransferase
2.4.1.38 beta-N-acetylglucosaminylglycopeptide beta-1,4-galactosyltransferase UDP-galactose:N-acetyl-beta-D-glucosaminylglycopeptide 4-beta-galactosyltransferase
2.4.1.90 N-acetyllactosamine synthase UDP-galactose:N-acetyl-D-glucosamine 4-beta-D-galactosyltransferase
2.8.2.21 keratan sulfotransferase 3'-phosphoadenylyl-sulfate:keratan 6'-sulfotransferase
3.1.6.14 N-acetylglucosamine-6-sulfatase N-acetyl-D-glucosamine-6-sulfate 6-sulfohydrolase
3.1.6.4 N-acetylgalactosamine-6-sulfatase N-acetyl-D-galactosamine-6-sulfate 6-sulfohydrolase


Pathway Source

Organism Taxonomy Group Enzyme List Source Ratio
Homo sapiens vertebrate_mammalian 2.4.1.- / 2.4.1.22 / 2.4.1.274 / 2.4.1.275 / 2.4.1.38 / 2.4.1.90 / 2.8.2.21 / 3.1.6.14 / 3.1.6.4 100.00%
Mus musculus vertebrate_mammalian 2.4.1.- / 2.4.1.22 / 2.4.1.274 / 2.4.1.275 / 2.4.1.38 / 2.4.1.90 / 2.8.2.21 / 3.1.6.14 / 3.1.6.4 100.00%
Rattus norvegicus vertebrate_mammalian 2.4.1.- / 2.4.1.22 / 2.4.1.274 / 2.4.1.275 / 2.4.1.38 / 2.4.1.90 / 2.8.2.21 / 3.1.6.14 / 3.1.6.4 100.00%
Saccharopolyspora erythraea NRRL 2338 MAGs 2.4.1.- / 3.1.6.14 22.22%
Aspergillus niger CBS 513.88 MAGs 2.4.1.- / 3.1.6.14 22.22%
Escherichia coli bacteria 2.4.1.- 11.11%
Bacillus anthracis bacteria 2.4.1.- 11.11%
Streptococcus parasanguinis bacteria 2.4.1.- 11.11%
Streptococcus gordonii bacteria 2.4.1.- 11.11%
Staphylococcus epidermidis bacteria 2.4.1.- 11.11%
Synechocystis sp. PCC 6803 bacteria 2.4.1.- 11.11%
Dichelobacter nodosus bacteria 2.4.1.- 11.11%
Shigella flexneri bacteria 2.4.1.- 11.11%
Neorhizobium galegae bacteria 2.4.1.- 11.11%
Rhizobium tropici bacteria 2.4.1.- 11.11%
Penicillium cf. griseofulvum MAGs 2.4.1.- 11.11%
Rhizobium sp. MPIK3030 MAGs 2.4.1.- 11.11%
Rhizobium leguminosarum bv. viciae MAGs 2.4.1.- 11.11%
Rhizobium leguminosarum bv. trifolii MAGs 2.4.1.- 11.11%
Rhizobium leguminosarum bv. phaseoli MAGs 2.4.1.- 11.11%