|N-acetylglucosaminidase, alpha- (Sanfilippo disease IIIB)|
|Locus||Chr. 17 q11-q21|
Alpha-N-acetylglucosaminidase (EC 184.108.40.206, 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 system name alpha-N-acetyl-D-glucosaminide N-acetylglucosaminohydrolase. 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.
- von Figura, K. (1977). "Human α-N-acetylglucosaminidase. 1. Purification and properties". Eur. J. Biochem. 80: 523–533. PMID 411658.
- von Figura, K. (1977). "Human α-N-acetylglucosaminidase. 2. Activity towards natural substrates and multiple recognition forms". Eur. J. Biochem. 80: 535–542. PMID 923593.
- Weissmann, B., Rowen, G., Marshall, J. and Friederici, D. (1967). "Mammalian α-acetylglucosaminidase. Enzymic properties, tissue distribution, and intracellular localization". Biochemistry 6: 207–214. PMID 4291567.
- Werries, E., Wollek, E., Gottschalk, A. and 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. PMID 5348072.
- alpha-N-acetyl-D-glucosaminidase at the US National Library of Medicine Medical Subject Headings (MeSH)
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