N-acetylgalactosamine-6-sulfatase

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.

N-acetylgalactosamine-6-sulfatase
Identifiers
EC no.3.1.6.4
CAS no.9025-60-9
Databases
IntEnzIntEnz view
BRENDABRENDA entry
ExPASyNiceZyme view
KEGGKEGG entry
MetaCycmetabolic pathway
PRIAMprofile
PDB structuresRCSB PDB PDBe PDBsum
Gene OntologyAmiGO / QuickGO
Search
PMCarticles
PubMedarticles
NCBIproteins

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
  1. Epstein EH, Leventhal ME (1981). "Steroid sulfatase of human leukocytes and epidermis and the diagnosis of recessive X-linked ichthyosis". J. Clin. Invest. 67 (5): 1257–62. doi:10.1172/JCI110153. PMC 370691. PMID 6939689.
  2. Sorensen SH, Noren O, Sjostrom H, Danielsen EM (1982). "Amphiphilic pig intestinal microvillus maltase/glucoamylase Structure and specificity". Eur. J. Biochem. 126 (3): 559–68. doi:10.1111/j.1432-1033.1982.tb06817.x. PMID 6814909.
  3. Lim CT, Horwitz AL (1981). "Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase". Biochim. Biophys. Acta. 657 (2): 344–55. doi:10.1016/0005-2744(81)90320-x. PMID 7213753.
  4. Glossl J, Kresse H (1982). "Impaired degradation of keratan sulphate by Morquio A fibroblasts". Biochem. J. 203 (1): 335–8. PMC 1158229. PMID 6213226.
  5. Yutaka T, Okada S, Kato T, Inui K, Yabuuhi H (1982). "Galactose 6-sulfate sulfatase activity in Morquio syndrome". Clin. Chim. Acta. 122 (2): 169–80. doi:10.1016/0009-8981(82)90276-5. PMID 6809361.


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