| methylumbelliferone sulfatase | <enzyme> Used as test substrate for arylsulfatases Registry number: EC 3.1.6.- Synonym: 4-methylumbelliferone sulfate sulfatase (26 Jun 1999) |
|---|---|
| placental sulfatase deficiency | <enzyme> An enzyme defect in the placenta which results in failure of conversion of 16a-hydroxydehydroepiandrosterone to estriol; women with this condition rarely enter into spontaneous labour. (05 Mar 2000) |
| multiple sulfatase deficiency | An inherited disorder (autosomal recessive) in which there is a failure to hydrolyze sulfatides and sulfated mucopolysaccharides; this failure leads to their accumulation in neural and extraneural tissues causing demyelination, sulfatiduria, facial and skeletal dysmorphism, etc. (05 Mar 2000) |
| N-acetylgalactosamine-6-sulfatase | <enzyme> Defective in mucopolysaccharidosis iva (morquio a) Registry number: EC 3.1.6.4 Synonym: n-acetylgalactosamine-6-sulfate sulfatase (26 Jun 1999) |
| N-acetylglucosamine-6-sulfatase | <enzyme> Deficient in sanfilippo syndrome type d Registry number: EC 3.1.6.14 Synonym: nagss, nagsase, glucosamine-6-sulfatase, n-acetylglucosamine-6-sulfate sulfatase (26 Jun 1999) |
| steroid sulfatase deficiency | A form of ichthyosis, due to 3-beta-hydroxysteroidsulfate sulfatase deficiency, that appears at birth or in early infancy and affects males; characterised by scaling predominantly on the neck and trunk but not on the palms and soles; histologically, there is hyperkeratosis, a granular layer in the epidermis, and normal epidermal cell turnover. Synonym: steroid sulfatase deficiency. (05 Mar 2000) |
| steryl-sulfatase | <enzyme> Arylsulfatases a and b hydrolyze nitrocatechol sulfate, but p-nitrophenyl sulfate is hydrolyzed only slightly by both enzymes; arylsulfatase c hydrolyzes both p-nitrophenyl sulfate and nitrocatechol sulfate, low substrate specificity located chiefly in microsomes; a and b are found in lysosomes; arylsulfatase a is cerebroside sulfatase and arylsulfatase b is chondro-4-sulfatase Registry number: EC 3.1.6.2 Synonym: arylsulfatase c, dehydroepiandrosterone sulfate sulfatase, steroid sulfatase, estrone sulfate sulfohydrolase, steroid sulphatase, cholesterol sulfatase, dhea sulfatase, 3 beta-hydroxysteroid sulfate sulfatase, estrone sulfate sulfatase, sterylsulfatase, steroid sulfohydrolase, cholesterol sulfate sulfatase, arylsulphatase c (26 Jun 1999) |
| N-sulfoglucosamine-3-sulfatase | <enzyme> Acts on n-acetylglucosamine-3-sulfate and n-sulfoglucosamine-3-sulfate Registry number: EC 3.1.6.15 Synonym: n-substituted glucosamine 3-o-sulphatase (26 Jun 1999) |
| sulfatase | 1. Trivial name for enzymes in EC group 3.1.6, the sulfuric ester hydrolases, which catalyze the hydrolysis of sulfuric esters (sulfates) to the corresponding alcohols plus inorganic sulfate; includes aryl-, sterol, glycol-, chondroitin, choline-, cellulose, cerebroside, and chondro-sulfatases. Synonym: arylsulfatase. (05 Mar 2000) |
| disulfoglucosamine-6-sulfatase | <enzyme> Acts on various n-substituted glucosamine-6-o-sulfates Registry number: EC 3.1.6.11 Synonym: disulphoglucosamine-6-sulphatase, glucosamine o,n-disulfate o-sulfohydrolase, glucosamine sulfatase, n-sulfoglucosamine-6-sulfatase (26 Jun 1999) |
| iduronate sulfatase | <enzyme> An enzyme that specifically cleaves the ester sulfate of iduronic acid, and is required for the desulfation of 2-sulfate iduronate residues in heparan sulfate. It is also required in dermatan sulfate degradation. Its deficiency has been demonstrated in Hunter's syndrome, which is characterised by an excess of dermatan sulfate and heparan sulfate. Chemical name: L-Iduronate-2-sulfate 2-sulfohydrolase Registry number: EC 3.1.6.13 (20 Sep 2002) |
| oligosaccharide 2-O-sulfatase | <enzyme> Hydrolyzes the 2-o-sulfate of the disaccharide 4-deoxy-2-o-sulfato-alpha-l-threo-hex-4-enopyranosyluronic acid-(1-4)-2-deoxy-2-sulfamido-6-o-sulfato-d-glucose from heparin Registry number: EC 3.1.6.- Synonym: flavobacterium heparinum 2-o-sulphatase, heparo-2-o-sulfatase (26 Jun 1999) |
| oligosaccharyl sulfatase | <enzyme> From keyhole limpet; hydrolyzes sulfate esters in asparagine-linked oligosaccharides preferentially to 4-nitrocatechol sulfate Registry number: EC 3.1.6.- (26 Jun 1999) |
| acid sulfate | A salt containing HSO4-. Synonym: acid sulfate. (05 Mar 2000) |
| active sulfate | 3'-phosphoadenosine 5'-phosphosulfate;an intermediate in the formation of urinary ethereal sulfates, notable for containing a "high energy" sulfate bond; the 3'-OH of adenosine is replaced by -OPO3H2, the 5'-OH by -OP(O2H)-OSO3H. Synonym: active sulfate. Acronym: PAPS (05 Mar 2000) |