Domain

Alpha-N-acetylglucosaminidase, tim-barrel domain (IPR024733)

Short name: NAGLU_tim-barrel

Domain relationships

None.

Description

Alpha-N-acetylglucosaminidase is a lysosomal enzyme that is required for the stepwise degradation of heparan sulphate [PMID: 10588735]. Mutations on the alpha-N-acetylglucosaminidase 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].

Alpha-N-acetylglucosaminidase is composed of three domains. This entry represents the central domain, which has a tim barrel fold [PMID: 18443291].

Contributing signatures

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