EC number 3.1.6.4 IntEnz IntEnz view ExPASy NiceZyme view | CAS number 9025-60-9 BRENDA BRENDA entry KEGG KEGG entry | |
In enzymology, a N-acetylgalactosamine-6-sulfatase (EC 3.1.6.4) is an enzyme that 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. Treatment options include enzyme replacement therapy with a synthetic version of the enzyme called elosulfase alfa.