Arylsulfatase B

MIM 253200) Sly (MIM 253220) MPS VII sulfatase (arylsulfatase B) P-Glucuronidase Dermatan sulfate, heparan sulfate, chondroitin... 

EC 3.1.6.1) is a lysosomal enzyme that hydrolyzes sulfuric acid ester bonds. The enzyme exists in two forms, arylsulfatases A and B, that differ in substrate specificity and in sensitivity toward inhibitors [142][143]. Human tissues contain more arylsulfatase A than arylsulfatase B. The natural substrates of these enzymes are complex lipids such as cerebroside 3-sulfate, and gly-cosaminoglycans such as chondroitin 4-sulfate and derman sulfate [144], Deficiencies of these enzymes are associated with a number of lysosomal disorders. 

The activity is calculated using the standard curve and the result for the absorption (patient) because during the first 30 min of incubation some of the activity may be derived from arylsulfatase A instead of arylsulfatase B. Results are expressed in nmol/ mg protein-h. 

Arylsulfatase B catalyzes the hydrolysis of the sulfate from UDP-Gal-NAc-4-sulfate to form UDP-GalNAc and sulfate. This activity is found in normal fibroblasts but not in Maroteaux-Lamy fibroblasts. 

Figure 9.65 HPLC analysis of arylsulfatase B reaction with UDP-GalNAc-4-S The reaction mixture contained 5 mU of enzyme. (From Fluharty et al., 1982).

MPS VI Maroteaux-Lamy Dysostosis multiplex, corneal clouding, normal intelligence survival to teens in severe form milder forms known to exist V-Acetylgalactosamine 4-sulfatase (arylsulfatase B) Dermatan sulfate... 

Arylsulfatase B (N-acetylgalactosamine 4-sulfatase) is a deficient enzyme in patients witti the genetic defect disease MPS VI. Like MPS I, GAGs are only partially broken down, and carbohydrate residues build relentlessly in the lysosomesof cells. Also like a-L-iduroni-dase replacement, the exogenous supply of arylsulfatase could breakdown the stored GAGs and cellular function could be restored. 

Aryplase is a specific form of the recombinant human enzyme, arylsulfatase B. In a phase I trial, the enzyme was well tolerated, and there were no drug-related serious adverse events and no significant allergic reactions to the infusions. Urinary GAG excretion was reduced by a mean of 70%in the high dose group and 55% in the low dose group. Urinary GAj excretion is a biomarker for MPS. 

Ho T, Maguire A, Aguirre G, Salvetti A, Haskins M, Bennett J. Phenotypic rescue after adeno-associated virus (AAV)-mediated delivery of arylsulfatase B (ASB) to the retinal pigment epithelium (RPE) of feline mucopolysaccharidosis VI (MPS VI). J Gene Med 2002 4 613-621. 

Farooqui, A. A., Effect of neurominidase on the multiple forms of arylsulfatase B. Biochlmie 58, 759-761 (1976b). 

Farooqui, A. A., Evidence of an essential histidyl residue in arylsulfatase B. Experientia 32, 1377-1379 (1976c). 

Fluharty, A. L., Stevens, R. L., Fung, D., Peak, S., and Kihara, H., UDP-N-Acetylgalac-tosamine 4-suUate sulfohydrolase activity of human arylsulfatase B and its deficiency in Maroteaux-Lamy syndrome. Biochem. Biophys. Res. Commun. 64, 955-962 (1975). 

Fluharty, A. L., Click, J. A., Samaan, G. F., and Kihara, H., Uridine diphospho-iV-acetylgalac-tosamine 4-sulfate Isolation and HPLC assay of its reaction with arylsulfatase B. Anal. Biochem. 121, 310-314 (1982). 

Gasa, S., and Makita, A. Phosphorylation on protein and carbohydrate moieties of a lysosomal arylsulfatase B variant in human lung cancer transplanted into athymic mice. J. Biol. Chem. 258, 5034-5039 (1983). 

Gasa, S., Makita, A., Kameya, T., Kodama, T., Koide, T., Tsumuraya, M., and Komai, T., Arylsul tases of human-lung tumors transplanted into alythmic mice—Cancer assoeiated modification of arylsulfatase B variant. Eur. J. Biochem. 116, 497-503 (1981). 

Gorham, S. D., and Gantz, M., Arylsulfatase B, an exosulfatase for chandroitin 4-sulfate tetrasaccharide. Hoppe-Seyler s Z. Physiol. Chem. 359, 1811-1814 (1978). 

Gniot-Szulzycka, J., Human placenta arylsulfatase B Purification and separation into subfractions. Acta Biochim. Pol. 19, 181-189 (1972). 

Lakshmi, S., and Balasubramanian, A. S., Soluble arylsulfatases of human brain and some characteristics of the brain specific arylsulfatase B ,. Biochim. Biophys. Acta 614, 446-458... 

Mathew, J., and Balasubramanian, A. S., Anionic forms ofbrain arylsulfatase B Evidence fora phosphorylated form in man and monkey. Dev. Neurosci. 6, 278-284 (1984). 

Steckel, F., Hasilik, A., and Von Figura, K., Biosynthesis and maturation of arylsulfatase B in normal and mutant cultured human fibroblasts. J. Biol. Chem. 258, 14322-14326 (1983). 

Uehara, Y., Casa, S., Makita, A., Sakurada, K., and Miyazaki, T., Expression of arylsulfatase B variant from leukocytes in chronic myelogenous leukemia related to chemotherapy. Tohoku ]. Exp. Med. 154, 311-319 (1985). 

Weller, P. F., Corey, E. J., Austen, K. F., and Lewis, R. A., Inhibition ofhomogenous human eosinophil arylsulfatase B by sulfidopeptide leukotrienes. J. Biol. Chem. 261, 1737—1744... 

Selvidge, L. A., and Verlangieri, A. J., 1991, Inhibition of arylsulfatase B by ascorbic acid. Res. Commun. Chem. Pathol. Pharmacol. 73 253-256. 

Laboratory findings are characterized by increased urinary excretion of mucopolysaccharides (Dorfman and Lorincz 1957), with values of 60 mg per liter and more as compared to normal values of 5—10 mg per liter. The urinary mucopolysaccharides in HD consist of chondroitinsulfate B and heparitin sulfate (Dorfman and Lorincz 1957, Meyer et al. 1958) with a 2 1 ratio. The occurrence of both mucopolysaccharides is used for distinction of HD from other mucopolysaccharidoses or other disorders associated with increased mucopolysaccharide excretion, such as the Marfan syndrome (Berenson and Dalferes 1965). The finding of a smaller proportion of heparitinsulfate in HD as compared to the sex-linked mucopolysaccharidosis II (see table 8) (Terry and Linker 1964) is not obligatory. Increased arylsulfatase B activity was found in the urine of patients with HD by Austin et al. (1964). 

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