| G6S | glucosamine-6-sulfatase |
|---|---|
| IDS | iduronate sulfatase; immune deficiency state; inhibitor of DNA synthesis; integrated delivery system... |
| MSD | material safety data; mean square deviation; mild sickle cell disease; most significant digit; multi... |
| SIDS | sudden infant death syndrome; sulfo-iduronate sulfatase |
| SSDD | steroid sulfatase deficiency disease |
| CST | Cerebroside sulfotransferase |
|---|---|
| CBS | cerebroside sulfate |
| ARSA | Aryl sulfatase A |
| IDS | Iduronate sulfatase |
| MSD | Multiple Sulfatase Deficiency |
| 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) |
|---|---|
| cerebroside | <biochemistry> Glycolipid found in brain (11% of dry matter). Sphingosine core with fatty amide or hydroxy fatty amide and a single monosaccharide on the alcohol group (either glucose or galactose). (18 Nov 1997) |
| cerebroside lipidosis | <disease> A chronic congenital disease of lipid metabolism caused by a deficiency of the beta-glucocerebrosidase enzyme. The defect is most common in Ashkenazi Jews. Clinical features are hepatosplenomegaly (enlargement of liver and spleen) and in severe early onset forms of the disease, with neurological dysfunction. Inheritance: autosomal recessive. (27 Sep 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) |
| 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) |
| 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) |
| 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) |
| heparan sulfate sulfatase | <enzyme> Probable defect in sanfilippo a syndrome Registry number: EC 3.1.6.- Synonym: heparan sulphatase (26 Jun 1999) |
| 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) |
| cerebroside sulfatase |
[EC 3.1.6.8] an enzyme of the hydrolase class that catalyzes the cleavage of sulfate residues from sulfatides to form cerebrosides. Deficiency of the enzyme, an autosomal recessive trait, is one of the causes of metachromatic leukodystrophy. Called also arylsulfatase A.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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