N-acetylglucosamine-6-sulfatase, metazoan (IPR015981)

Short name: GlcNAc_6-SO4ase_met

Overlapping homologous superfamilies

Family relationships


N-acetylglucosamine-6-sulphatase catalyses the hydrolysis of the 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of heparan sulfate and keratan sulfate [PMID: 3689315]. This enzyme is deficient in Sanfilippo Syndrome type IIID [PMID: 2500866]. Mucopolysaccharidosis type III (MPS-III), or Sanfilippo syndrome, is a group of lysosomal storage disorders caused by impaired degradation of heparan sulfate.

GO terms

Biological Process

GO:0030203 glycosaminoglycan metabolic process

Molecular Function

GO:0008449 N-acetylglucosamine-6-sulfatase activity

Cellular Component

GO:0005764 lysosome

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.