N-acetylgalactosamine-6-sulfatase explained

N-acetylgalactosamine-6-sulfatase
Ec Number:	3.1.6.4
Cas Number:	9025-60-9
Go Code:	0043890

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.

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