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N-acetylgalactosamine-6-sulfatase

The enzyme N-acetylgalactosamine-6-sulfatase (EC 3.1.6.4) catalyzes the chemical reaction of cleaving off the 6-sulfate groups of the N-acetyl-<small>D</small>-galactosamine 6-sulfate units of the macromolecule chondroitin sulfate and, similarly, of the <small>D</small>-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-<small>D</small>-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.

References