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InterPro: IPR007781 Alpha-N-acetylglucosaminidase

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Matches:
151 proteins
AccessionHelp IPR007781 GlcNAc_a
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Alpha-N-acetylglucosaminidase is a lysosomal enzyme required for the stepwise degradation of heparan sulphate [1]. 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 [2].

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PANDIT: PF05089

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Example proteinsHelp
P54802 Alpha-N-acetylglucosaminidase

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InterPro entry accession number/name and structure databases Colour code
IPR007781 Alpha-N-acetylglucosaminidase
SWISS-MODEL
ModBase

PublicationsHelp
1. Li HH, Yu WH, Rozengurt N, Zhao HZ, Lyons KM, Anagnostaras S, Fanselow MS, Suzuki K, Vanier MT, Neufeld EF.
Mouse model of Sanfilippo syndrome type B produced by targeted disruption of the gene encoding alpha-N-acetylglucosaminidase.
Proc. Natl. Acad. Sci. U.S.A. 96 14505-10 1999 [PubMed: 10588735]
http://dx.doi.org/10.1073/pnas.96.25.14505
2. Villani GR, Follenzi A, Vanacore B, Di Domenico C, Naldini L, Di Natale P.
Correction of mucopolysaccharidosis type IIIb fibroblasts by lentiviral vector-mediated gene transfer.
Biochem. J. 364 747-53 2002 [PubMed: 12049639]
http://dx.doi.org/10.1042/BJ20011872

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InterPro 23.1