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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 (November 1977). "Human alpha-N-acetylglucosaminidase. 1. Purification and properties". European Journal of Biochemistry. 80 (2): 523–33. doi:10.1111/j.1432-1033.1977.tb11908.x. PMID 411658. 
  2. ^ von Figura K (November 1977). "Human alpha-n-acetylglucosaminidase. 2. Activity towards natural substrates and multiple recognition forms". European Journal of Biochemistry. 80 (2): 535–42. doi:10.1111/j.1432-1033.1977.tb11909.x. PMID 923593. 
  3. ^ Weissmann B, Rowin G, Marshall J, Friederici D (January 1967). "Mammalian alpha-acetylglucosaminidase. Enzymic properties, tissue distribution, and intracellular localization". Biochemistry. 6 (1): 207–14. doi:10.1021/bi00853a033. PMID 4291567. 
  4. ^ Werries E, Wollek E, Gottschalk A, Buddecke E (October 1969). "Separation of N-acetyl-alpha-glucosaminidase and N-acetyl-alpha-galactosaminidase from ox spleen. Cleavage of the O-glycosidic linkage between carbohydrate and polypeptide in ovine and bovine submaxillary glycoprotein by N-acetyl-alpha-galactosaminidase". European Journal of Biochemistry. 10 (3): 445–9. doi:10.1111/j.1432-1033.1969.tb00709.x. PMID 5348072. 

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