Glycosaminoglycan metabolism

Clarke LA. The mucopolysaccharidoses: a success of molecular medicine. Expert Rev Mol Med. 2008 Jan 18;10():e1. doi: 10.1017/s1462399408000550. PMID: 18201392.; Cantz M, Gehler J. The mucopolysaccharidoses: inborn errors of glycosaminoglycan catabolism. Hum Genet. 1976 Jun 29;32(3):233–55. doi: 10.1007/bf00295816. PMID: 820626.

Metabolites

alpha-D-Galactose

Formula: C6H12O6 (180.0633852)

CAS ID: 3646-73-9

Calcium cation

Formula: Ca (39.962591)

CAS ID: 14127-61-8

CO2

Formula: CO2 (43.98983)

CAS ID: 124-38-9

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

D-glucopyranuronate

Formula: C6H9O7 (193.0348264)

CAS ID: 148-00-5

L-aspartate(1-)

Formula: C4H6NO4 (132.0296816)

CAS ID:

acetate

Formula: C2H3O2 (59.0133038)

CAS ID: 71-50-1

ATP(4-)

Formula: C10H12N5O13P3 (502.9644492)

CAS ID:

UDP-N-acetyl-D-galactosamine(2-)

Formula: C17H25N3O17P2 (605.0659180000001)

CAS ID:

NAD(1-)

Formula: C21H26N7O14P2 (662.1012936000001)

CAS ID: 53-84-9

UDP(3-)

Formula: C9H11N2O12P2 (400.9787246)

CAS ID: 86527-70-0

ADP(3-)

Formula: C10H12N5O10P2 (424.0059412)

CAS ID:



Enzyme

EC Number name full name note
2.7.1.-
2.4.1.133 xylosylprotein 4-beta-galactosyltransferase UDP-galactose:O-beta-D-xylosylprotein 4-beta-D-galactosyltransferase
2.4.1.135 galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase UDP-alpha-D-glucuronate:[protein]-3-O-(beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl)-L-serine D-glucuronosyltransferase (configuration-inverting)
2.4.1.223 glucuronosyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase UDP-N-acetyl-alpha-D-glucosamine:[protein]-3-O-(beta-D-GlcA-(1->3)-beta-D-Gal-(1->3)-beta-D-Gal-(1->4)-beta-D-Xyl)-L-serine 4IV-alpha-N-acetyl-D-glucosaminyltransferase (configuration-retaining)
2.4.1.224 glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase UDP-N-acetyl-D-glucosamine:beta-D-glucuronosyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
2.4.1.225 N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase UDP-alpha-D-glucuronate:N-acetyl-alpha-D-glucosaminyl-(1->4)-beta-D-glucuronosyl-proteoglycan 4-beta-glucuronosyltransferase
2.4.1.244 N-acetyl-beta-glucosaminyl-glycoprotein 4-beta-N-acetylgalactosaminyltransferase UDP-N-acetyl-D-galactosamine:N-acetyl-beta-D-glucosaminyl-group 4-beta-N-acetylgalactosaminyltransferase
2.4.2.26 protein xylosyltransferase UDP-alpha-D-xylose:protein beta-D-xylosyltransferase (configuration-inverting)
2.8.2.8 [heparan sulfate]-glucosamine N-sulfotransferase 3'-phosphoadenylyl-sulfate:[heparan sulfate]-glucosamine N-sulfotransferase
3.2.1.76 L-iduronidase glycosaminoglycan alpha-L-iduronohydrolase


Proteins

Protein ID name full name