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

Magnesium cation

Formula: Mg (23.98505)

CAS ID: 22537-22-0

Manganese(2+)

Formula: Mn (54.938046)

CAS ID: 7439-96-5

N-Acetyl-D-galactosamine

Formula: C8H15NO6 (221.089933)

CAS ID: 14215-68-0

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-alpha-D-glucose(2-)

Formula: C15H22N2O17P2 (564.0393702)

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:

histaminium

Formula: C5H10N3 (112.087468)

CAS ID: 65592-96-3



Enzyme

EC Number name full name note
2.4.1.-
2.4.1.133 xylosylprotein 4-beta-galactosyltransferase UDP-galactose:O-beta-D-xylosylprotein 4-beta-D-galactosyltransferase
2.4.1.134 galactosylxylosylprotein 3-beta-galactosyltransferase UDP-galactose:4-beta-D-galactosyl-O-beta-D-xylosylprotein 3-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)
2.4.1.22 lactose synthase UDP-galactose:D-glucose 4-beta-D-galactosyltransferase
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
2.4.1.274 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
2.8.2.23 [heparan sulfate]-glucosamine 3-sulfotransferase 1 3'-phosphoadenylyl-sulfate:[heparan sulfate]-glucosamine 3-sulfotransferase
2.8.2.33 N-acetylgalactosamine 4-sulfate 6-O-sulfotransferase 3'-phosphoadenylyl-sulfate:[dermatan]-4-O-sulfo-N-acetyl-D-galactosamine 6-O-sulfotransferase
2.8.2.35 dermatan 4-sulfotransferase 3'-phospho-5'-adenylyl sulfate:[dermatan]-N-acetyl-D-galactosamine 4-sulfotransferase
3.1.6.12 N-acetylgalactosamine-4-sulfatase N-acetyl-D-galactosamine-4-sulfate 4-sulfohydrolase
3.1.6.13 iduronate-2-sulfatase L-iduronate-2-sulfate 2-sulfohydrolase
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
3.10.1.1 N-sulfoglucosamine sulfohydrolase N-sulfo-D-glucosamine sulfohydrolase
3.2.1.166 heparanase heparan sulfate N-sulfo-D-glucosamine endoglucanase
3.2.1.31 beta-glucuronidase beta-D-glucuronoside glucuronosohydrolase
3.2.1.35 hyaluronoglucosaminidase hyaluronate 4-glycanohydrolase
3.2.1.50 alpha-N-acetylglucosaminidase alpha-N-acetyl-D-glucosaminide N-acetylglucosaminohydrolase
3.2.1.76 L-iduronidase glycosaminoglycan alpha-L-iduronohydrolase
3.4.22.15 cathepsin L
4.1.1.35 UDP-glucuronate decarboxylase UDP-D-glucuronate carboxy-lyase (UDP-D-xylose-forming)
5.1.3.17 heparosan-N-sulfate-glucuronate 5-epimerase poly[(1->4)-beta-D-glucuronosyl-(1->4)-N-sulfo-alpha-D-glucosaminyl] glucurono-5-epimerase
7.6.2.2 ABC-type xenobiotic transporter ATP phosphohydrolase (ABC-type, xenobiotic-exporting)


Proteins

Protein ID name full name