Heparan sulfate/heparin (HS-GAG) metabolism

Ballabio A, Gieselmann V. Lysosomal disorders: From storage to cellular damage. Biochimica et Biophysica Acta (BBA) - Molecular Cell Research. 2009 Apr;1793(4):684–96. doi: 10.1016/j.bbamcr.2008.12.001.; Sasisekharan R, Venkataraman G. Heparin and heparan sulfate: biosynthesis, structure and function. Curr Opin Chem Biol. 2000 Dec;4(6):626–31. doi: 10.1016/s1367-5931(00)00145-9. PMID: 11102866.; Tumova S, Woods A, Couchman JR. Heparan sulfate proteoglycans on the cell surface: versatile coordinators of cellular functions. The International Journal of Biochemistry & Cell Biology. 2000 Mar;32(3):269–88. doi: 10.1016/s1357-2725(99)00116-8.; Stringer SE, Gallagher JT. Heparan sulphate. The International Journal of Biochemistry & Cell Biology. 1997 May;29(5):709–14. doi: 10.1016/s1357-2725(96)00170-7.

Metabolites

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

acetate

Formula: C2H3O2 (59.0133038)

CAS ID: 71-50-1

UDP(3-)

Formula: C9H11N2O12P2 (400.9787246)

CAS ID: 86527-70-0



Enzyme

EC Number name full name note
2.8.2.-
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
2.8.2.23 [heparan sulfate]-glucosamine 3-sulfotransferase 1 3'-phosphoadenylyl-sulfate:[heparan sulfate]-glucosamine 3-sulfotransferase
3.1.6.13 iduronate-2-sulfatase L-iduronate-2-sulfate 2-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
3.2.1.50 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
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


Proteins

Protein ID name full name