| heparan sulfate sulfatase | <enzyme> Probable defect in sanfilippo a syndrome Registry number: EC 3.1.6.- Synonym: heparan sulphatase (26 Jun 1999) |
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| heparan N-sulfatase | <enzyme> An enzyme that participates in the stepwise degradation of heparan sulfate; heparan N-sulfatase hydrolyzes the sulfate moiety attached to the amino group of the glucosamine residue of heparan sulfate; a deficiency of this enzyme is associated with mucopolysaccharidose IIIA (Sanfilippo's syndrome A). (05 Mar 2000) |
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| heparan sulfate | <chemical> Heparitin, sulfate. A glycosaminoglycan occurring in the liver, aorta, and lung. It has a structure similar to heparin, but there are more n-acetyl groups and fewer o- and n-sulfate groups. It is an accumulation product in several mucopolysaccharidoses. Chemical name: Heparitin, sulfate (12 Dec 1998) |
| heparan-sulfate 2-sulfotransferase | <enzyme> An o-sulfotransferase that catalyses transfer of sulfate from 3'-phosphoadenosine 5'-phosphosulfate to l-iduronic acid at position 2 of heparan sulfate; genbank d88811 Registry number: EC 2.8.2.- Synonym: hs2st enzyme (26 Jun 1999) |
| heparan sulfate D-glucosaminyl 3-O-sulfotransferase | <enzyme> Involved in biosynthesis of heparan sulfate proteoglycans Registry number: EC 2.8.2.- Synonym: hsg 3-o-sulfotransferase (26 Jun 1999) |
| heparan sulfate-N-deacetylase-N-sulfotransferase | <enzyme> Catalyses both the n-deacetylation and the n-sulfation reactions that begin modification of heparan sulfate's oligosaccharide backbone Registry number: EC 2.8.2.- Synonym: hs-ndac-nst (26 Jun 1999) |
| heparan sulfate proteoglycan | Ubiquitous macromolecules associated with the cell surface and extracellular matrix of a wide range of cells of vertebrate and invertebrate tissues. They are essential cofactors in cell-matrix adhesion processes, in cell-cell recognition systems, and in receptor-growth factor interactions. (12 Dec 1998) |
| lactate-2-sulfate sulfatase | <enzyme> Shows no activity for (l)-isomer Registry number: EC 3.1.6.- Synonym: d-lactate-2-sulfate sulfatase, d-lactate-2-sulfatase (26 Jun 1999) |
| 3b-hydroxysteroid sulfate sulfatase | <enzyme> An enzyme, found in most mammalian tissues, that is capable of hydrolyzing the sulfate ester bonds of a variety of sulfated sterols; a deficiency of this enzyme will result in X-linked ichthyosis. (05 Mar 2000) |
| heparan sulphate | Constituent of membrane associated proteoglycans. The heparan sulphate binding domain of NCAM is proposed to augment NCAM NCAM interactions, suggesting that cell cell bonds mediated by NCAM may involve interactions between multiple ligands. The putative heparin binding site on NCAM is a 28 aa peptide shown to bind both heparin and retinal cells, as well as to inhibit retinal cell adhesion to NCAM. This strengthens the argument that that this site contributes directly to NCAM mediated cell cell adhesion. (18 Nov 1997) |
| bile salt sulfatase | <enzyme> Produces microorganism from the faecal flora of conventional rats Registry number: EC 3.1.6.- Synonym: bile acid sulfate sulfatase (26 Jun 1999) |
| galactose-6-sulfatase | <enzyme> An enzyme that eliminates sulfur from the galactose-6-sulfate residues of certain mucopolysaccharides, producing 3,6-anhydrogalactose residues; it is absent in Morquio's syndrome type A. Synonym: galactose-6-sulfurase. (05 Mar 2000) |
| glucuronate 2-sulfatase | <enzyme> does not act on iduronate-2-sulfate Registry number: EC 3.1.6.- Synonym: glucurono-2-sulfatase (26 Jun 1999) |
| cerebroside-sulfatase | <enzyme> An enzyme that catalyses the hydrolysis of cerebroside 3-sulfate (sulfatide) to yield a cerebroside and inorganic sulfate. A marked deficiency of arylsulfatase a, which is considered the heat-labile component of cerebroside sulfatase, has been demonstrated in all forms of metachromatic leukodystrophy (leukodystrophy, metachromatic). Chemical name: Cerebroside-3-sulfate 3-sulfohydrolase Registry number: EC 3.1.6.8 (12 Dec 1998) |
| chondro-2-sulfatase | <enzyme> Acts on d-glucuronate 2-sulfate units of chondroitin sulfate, heparin and heparan sulfate Registry number: EC 3.1.6.- (26 Jun 1999) |
| chondro-4-sulfatase | <enzyme> An arylsulfatase that catalyses the hydrolysis of the 4-sulfate groups of the disaccharide repeating units from chondroitin sulfate n-acetylgalactosamine 4-sulfate. The deficiency of chondro-4-sulfatase has been proposed as the defect in maroteaux-lamy syndrome (mucopolysaccharidosis vi). Chemical name: 4-Deoxy-beta-D-gluc-4-enuronosyl-(1,3)-N-acetyl-D-galactosamine-4-sulfate 4-sulfohydrolase Registry number: EC 3.1.6.9 (12 Dec 1998) |