N-acetylglucosaminidase, alpha- (Sanfilippo disease IIIB)
Symbol NAGLU
Entrez 4669
HUGO 7632
OMIM 609701
RefSeq NM_000263
UniProt P54802
Other data
EC number
Locus Chr. 17 q11-q21

Alpha-N-acetylglucosaminidase (EC, alpha-acetylglucosaminidase, N-acetyl-alpha-D-glucosaminidase, N-acetyl-alpha-glucosaminidase, alpha-D-2-acetamido-2-deoxyglucosidase) is a protein associated with Sanfilippo syndrome. It is an enzyme with systematic name alpha-N-acetyl-D-glucosaminide N-acetylglucosaminohydrolase.[1][2][3][4] This enzyme catalyses the following chemical reaction

Hydrolysis of terminal non-reducing N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides

This enzyme hydrolyses UDP-N-acetylglucosamine.


  1. von Figura, K. (1977). "Human α-N-acetylglucosaminidase. 1. Purification and properties". Eur. J. Biochem. 80: 523–533. doi:10.1111/j.1432-1033.1977.tb11908.x. PMID 411658.
  2. von Figura, K. (1977). "Human α-N-acetylglucosaminidase. 2. Activity towards natural substrates and multiple recognition forms". Eur. J. Biochem. 80: 535–542. doi:10.1111/j.1432-1033.1977.tb11909.x. PMID 923593.
  3. Weissmann, B.; Rowen, G.; Marshall, J.; Friederici, D. (1967). "Mammalian α-acetylglucosaminidase. Enzymic properties, tissue distribution, and intracellular localization". Biochemistry. 6: 207–214. doi:10.1021/bi00853a033. PMID 4291567.
  4. Werries, E.; Wollek, E.; Gottschalk, A.; Buddecke, E. (1969). "Separation of N-acetyl-α-glucosaminidase and N-acetyl-α-galactosaminidase from ox spleen. Cleavage of the O-glycosidic linkage between carbohydrate and polypeptide in ovine and bovine submaxillary glycoprotein by N-acetyl-α-galactosaminidase". Eur. J. Biochem. 10: 445–449. doi:10.1111/j.1432-1033.1969.tb00709.x. PMID 5348072.

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