Alpha-N-acetylglucosaminidase

N-acetylglucosaminidase, alpha- (Sanfilippo disease IIIB)
Identifiers
Symbol	NAGLU
Entrez	4669
HUGO	7632
OMIM	609701
RefSeq	NM_000263
UniProt	P54802
Other data
EC number	3.2.1.50
Locus	Chr. 17 q11-q21

Alpha-N-acetylglucosaminidase (EC 3.2.1.50, 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.

References

↑ 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.
↑ 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.
↑ Weissmann, B., Rowen, G., Marshall, J. and Friederici, D. (1967). "Mammalian α-acetylglucosaminidase. Enzymic properties, tissue distribution, and intracellular localization". Biochemistry 6: 207–214. doi:10.1021/bi00853a033. 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. doi:10.1111/j.1432-1033.1969.tb00709.x. PMID 5348072.

External links

alpha-N-acetyl-D-glucosaminidase at the US National Library of Medicine Medical Subject Headings (MeSH)

glycosaminoglycan anabolism

Heparan sulfate: EXT1; EXT2

Chondroitin sulfate: PAPSS1; PAPSS2

glycosaminoglycan catabolism

Hunter, Hurler	Iduronate-2-sulfatase Iduronidase

Sanfilippo, Sly	Heparan sulfamidase N-acetyltransferase Alpha-N-acetylglucosaminidase Glucuronidase N-acetylglucosamine-6-sulfatase

Morquio/Maroteaux-Lamy	Arylsulfatase B Galactosamine-6 sulfatase Beta-galactosidase (GLB1)

Hydrolase: sugar hydrolases (EC 3.2)

3.2.1: Glycoside hydrolases

Disaccharidase	Sucrase/Sucrase-isomaltase/Invertase Maltase Trehalase Lactase

Glucosidases	Cellulase Alpha-glucosidase Acid Neutral AB Neutral C Beta-glucosidase cytosolic Debranching enzyme

Other	Amylase Alpha-amylase Chitinase Lysozyme Neuraminidase NEU1 NEU2 NEU3 NEU4 Bacterial neuraminidase Viral neuraminidase Galactosidases Alpha Beta alpha-Mannosidase Glucuronidase Hyaluronidase Pullulanase Glucosylceramidase lysosomal non-lysosomal Galactosylceramidase Alpha-N-acetylgalactosaminidase NAGA Alpha-N-acetylglucosaminidase Fucosidase Hexosaminidase HEXA HEXB Iduronidase Maltase-glucoamylase Heparanase HPSE2

3.2.2: Hydrolysing
N-Glycosyl compounds

DNA glycosylases: Oxoguanine glycosylase

Enzymes

Activity	Active site Binding site Catalytic triad Oxyanion hole Enzyme promiscuity Catalytically perfect enzyme Coenzyme Cofactor Enzyme catalysis Enzyme kinetics Lineweaver–Burk plot Michaelis–Menten kinetics

Regulation	Allosteric regulation Cooperativity Enzyme inhibitor

Classification	EC number Enzyme superfamily Enzyme family List of enzymes

Types	EC1 Oxidoreductases(list) EC2 Transferases(list) EC3 Hydrolases(list) EC4 Lyases(list) EC5 Isomerases(list) EC6 Ligases(list)

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