Domain

Alpha-N-acetylglucosaminidase, N-terminal (IPR024240)

Short name: NAGLU_N

Domain relationships

None.

Description

Alpha-N-acetylglucosaminidase, is a lysosomal enzyme required for the stepwise degradation of heparan sulphate [PMID: 10588735]. Mutations on the alpha-N-acetylglucosaminidase (NAGLU) gene can lead to Mucopolysaccharidosis type IIIB (MPS IIIB; or Sanfilippo syndrome type B) characterised by neurological dysfunction but relatively mild somatic manifestations [PMID: 12049639]. The structure shows that the enzyme is composed of three domains.

This entry represents the N-terminal domain of Alpha-N-acetylglucosaminidase which has an alpha-beta fold [PMID: 18443291].

Contributing signatures

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