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


Proteins

Protein ID name full name