3.1.6.12: N-acetylgalactosamine-4-sulfatase
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For detailed information about N-acetylgalactosamine-4-sulfatase, go to the full flat file.
Word Map on EC 3.1.6.12
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3.1.6.12
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mucopolysaccharidosis
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lysosomal
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maroteaux-lamy
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glycosaminoglycans
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arylsulfatase
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dermatan
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feline
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rhasb
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medicine
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galsulfase
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sulfatases
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stair
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enzyme-replacement
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mps-vi
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dysostosis
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diagnostics
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nutrition
- 3.1.6.12
- mucopolysaccharidosis
- lysosomal
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maroteaux-lamy
- glycosaminoglycans
- arylsulfatase
- dermatan
- feline
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rhasb
- medicine
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galsulfase
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sulfatases
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stair
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enzyme-replacement
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mps-vi
- dysostosis
- diagnostics
- nutrition
Reaction
Synonyms
4-sulfatase, 4-sulphatase, acetylgalactosamine 4-sulfatase, ARSB, arylsulfatase B, ASB, chondroitinase, chondroitinsulfatase, chondrosulfatase, G4S, gastric chondrosulfohydrolase, N-acetylgalactosamine 4-sulfatase, N-acetylgalactosamine 4-sulfate sulfohydrolase, N-acetylgalactosamine-4-sulfatase, N-acetylgalactosamine-4-sulphatase, sulfatase, acetylgalactosamine 4-
ECTree
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Application on EC 3.1.6.12 - N-acetylgalactosamine-4-sulfatase
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APPLICATION 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
diagnostics
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altered ARSB immunostaining and reduced activity may be useful indicators of malignant transformation in human colonic tissue
medicine
nutrition
in the renal tissue of Dahl salt-sensitive rats exposed to high salt diet, arylsulfatase B activity is significantly less than in Dahl salt-sensitive rats exposed to low salt diet, and chondroitin-4-sulfate and total sulfated glycosaminoglycan content are significantly greater. A marked increase in chondroitin-4-sulfate disaccharidesis observed in the renal tissue of the rats exposed to high salt diet. Unsulfated, hyaluronan-derived disaccharides are increased in the rats on the low salt diet. In the rats on high salt diet, with lower arylsulfatase B activity and higher chondroitin-4-sulfate levels, cell-bound, high-molecular weight kininogen is greater and urinary bradykinin is lower. Arylsulfatase B activity in renal tissue and normal rat kidney cells declines when exogenous chloride concentration is increased in vitro
additional information
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a relatively high rate of immunotolerance towards recombinant human N-acetylgalactosamine-4-sulfatase can be achieved in MPS-VI cats with a shortcourse tolerisation regimen ultimately permitting removal of lysosomal storage within the dura mater with the use of intrathecal therapy
medicine
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deficiency in Maroteaux-Lamy syndrome, mucopolysaccharidosis type IV
medicine
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application of recombinant enzyme may give improvement in mucopolysaccheridosis VI
medicine
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deficiency of arylsulfatase B causes mucopolysaccharidosis VI (Maroteaux-Lamy Syndrome)
medicine
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mucopolysaccharidosis type VI is a lysosomal storage disease in which deficient activity of the enzyme arylsulfatase B impairs the stepwise degradation of the glycosaminoglycan dermatan sulfate
medicine
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intrathecal administration of recombinant human N-acetylgalactosamine 4-sulfatase to a MPS VI patient with pachymeningitis cervicalis, overview
medicine
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usage of ASB for enzyme replacement therapy in mucopolysaccharidosis type VI, overview
medicine
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in prostatic malignancy, activity of arylsulfatase B declines. Arylsulfatase B may be useful as a biomarker of prostate cancer. In 82% of paired cases, the biochemical recurrences show lower arylsulfatase B immunostaining at the time of prostatectomy. Arylsulfatase B immunostaining is inversely associated with Gleason scores for epithelial and stromal compartments separately and in combination. Arylsulfatase B activity is significantly less in the malignant compared to normal tissue. In association with reduced arylsulfatase B activity, total sulfated glycosaminoglycans and chondroitin-4-sulfate content are increased in the malignant prostatic tissue, and the chondroitin-4-sulfate containing matrix proteoglycan versican is also increased
medicine
in the renal tissue of Dahl salt-sensitive rats exposed to high salt diet, arylsulfatase B activity is significantly less than in Dahl salt-sensitive rats exposed to low salt diet, and chondroitin-4-sulfate and total sulfated glycosaminoglycan content are significantly greater. A marked increase in chondroitin-4-sulfate disaccharidesis observed in the renal tissue of the rats exposed to high salt diet. Unsulfated, hyaluronan-derived disaccharides are increased in the rats on the low salt diet. In the rats on high salt diet, with lower arylsulfatase B activity and higher chondroitin-4-sulfate levels, cell-bound, high-molecular weight kininogen is greater and urinary bradykinin is lower. Arylsulfatase B activity in renal tissue and normal rat kidney cells declines when exogenous chloride concentration is increased in vitro
medicine
administration of recombinant enzyme as 2- and 4-h intravenous infusions using a feline model of mucopolysaccharidosis VI does not lead to significant differences in clinical outcomes such as urinary glycosaminoglycan excretion, tissue glycosaminoglycan storage, tissue galsulfase activity, and beta-glucuronidase but all are significantly different to control animals
medicine
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application of a combined assay for defects in iduronate-2-sulfatase (ID2S) leading to mucopolysaccharidosis II, and N-acetylgalactosamine-6-sulfatase (GALN) and N-acetylgalactosamine-4-sulfatase (ARSB) defects related to mucopolysaccharidosis IVA and MPS VI, respectively. The average enzyme activities of ID2S, GALN, and ARSB in random neonates are 19.6, 1.7, and 13.4 micromol/h/l, respectively. The average enzyme activities of ID2S, GALN, and ARSB in disease-affected individuals are 0.5, 0.3, and 0.3 micromol/h/l, respectively
medicine
ARSB is reduced in cystic fibrosis cells and increases when defective cystic fibrosis transmembrane conductance regulator CFTR is repaired. The CFTR potentiator VRT-532 increases ARSB activity and expression to the level in normal bronchial epithelial cells. Concomitantly, total sulfated glycosaminoglycans and chondroitin 4-sulfate decline, secreted interleukin IL-8 increases, secreted IL-6 declines, and neutrophil chemotaxis to the spent media obtained from the potentiator-treated cystic fibrosis cells increases
medicine
in human prostate cancer tissues, the N-acetylgalactosamine-4-sulfatase ARSB activity is reduced and the galactosamine-N-acetyl-6-sulfatase GALNS activity is increased, compared to normal prostate tissue
