Biology:N-acetylgalactosamine-6-sulfatase
| N-acetylgalactosamine-6-sulfatase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| EC number | 3.1.6.4 | ||||||||
| CAS number | 9025-60-9 | ||||||||
| Databases | |||||||||
| IntEnz | IntEnz view | ||||||||
| BRENDA | BRENDA entry | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / QuickGO | ||||||||
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The enzyme N-acetylgalactosamine-6-sulfatase (EC 3.1.6.4)[1][2][3] catalyzes the chemical reaction of cleaving off the 6-sulfate groups of the N-acetyl-D-galactosamine 6-sulfate units of the macromolecule chondroitin sulfate and, similarly, of the D-galactose 6-sulfate units of the macromolecule keratan sulfate.
This enzyme belongs to the family of hydrolases, specifically those acting on sulfuric ester bonds. The systematic name of this enzyme class is N-acetyl-D-galactosamine-6-sulfate 6-sulfohydrolase. Other names in common use include chondroitin sulfatase, chondroitinase, galactose-6-sulfate sulfatase, acetylgalactosamine 6-sulfatase, N-acetylgalactosamine-6-sulfate sulfatase, and N-acetylgalactosamine 6-sulfatase. This enzyme participates in glycosaminoglycan degradation and degradation of glycan structures.
Deficiency
Morquio syndrome is a rare birth defect caused by a deficiency in this essential enzyme.[4][5] Treatment options include enzyme replacement therapy with a synthetic version of the enzyme called elosulfase alfa.
References
- ↑ "Steroid sulfatase of human leukocytes and epidermis and the diagnosis of recessive X-linked ichthyosis". J. Clin. Invest. 67 (5): 1257–62. 1981. doi:10.1172/JCI110153. PMID 6939689.
- ↑ "Amphiphilic pig intestinal microvillus maltase/glucoamylase Structure and specificity". Eur. J. Biochem. 126 (3): 559–68. 1982. doi:10.1111/j.1432-1033.1982.tb06817.x. PMID 6814909.
- ↑ "Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase". Biochim. Biophys. Acta 657 (2): 344–55. 1981. doi:10.1016/0005-2744(81)90320-x. PMID 7213753.
- ↑ "Impaired degradation of keratan sulphate by Morquio A fibroblasts". Biochem. J. 203 (1): 335–8. 1982. PMID 6213226.
- ↑ "Galactose 6-sulfate sulfatase activity in Morquio syndrome". Clin. Chim. Acta 122 (2): 169–80. 1982. doi:10.1016/0009-8981(82)90276-5. PMID 6809361.
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