N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase is an
enzyme
Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products. A ...
that in humans is encoded by the ''AGA''
gene
In biology, the word gene (from , ; "...Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a ba ...
.
Aspartylglucosaminidase is an
amidohydrolase Amidohydrolases (or amidases) are a type of hydrolase that acts upon amide bonds.
They are categorized under EC number EC 3.5.1 and 3.5.2.
Examples include:
* Beta-lactamase
* Histone deacetylase
* Urease
Ureases (), functionally, belong to t ...
enzyme
Enzymes () are proteins that act as biological catalysts by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products. A ...
involved in the catabolism of
N-linked oligosaccharides of
glycoproteins
Glycoproteins are proteins which contain oligosaccharide chains covalently attached to amino acid side-chains. The carbohydrate is attached to the protein in a cotranslational or posttranslational modification. This process is known as glycos ...
. It cleaves asparagine from N-acetylglucosamines as one of the final steps in the lysosomal breakdown of glycoproteins. The lysosomal storage disease
aspartylglycosaminuria is caused by a deficiency in the AGA enzyme.
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{{hydrolase-stub