aspartylglucosaminidase (N-aspartyl-beta-glucosaminidase, N4-(beta-N-acetylglucosaminyl)-L-asparaginase, glycosylasparaginase, [contains: glycosylasparaginase alpha chain; glycosylasparaginase beta chain], AGA)

Function

• cleaves the GlcNAc-Asn bond which joins oligosaccharides to the peptide of asparagine-linked glycoproteins

N(4)-(beta-N-acetyl-D-glucosaminyl)-L- asparagine + H2O

<-->

N-acetyl-beta-D-glucosaminylamine + L-aspartate

Structure

• heterotetramer of two alpha & two beta chains
• belongs to the asparaginase 2 family

Compartment

Lysosome

Pathology

• defects in AGA are the cause of aspartylglucosaminuria

Laboratory

• aspartylglucosaminidase in fibroblasts
• aspartylglucosaminidase in leukocytes

More general terms

• amidohydrolase
• lysosomal hydrolase

Database

• UniProt: http://www.uniprot.org/uniprot/P20933.html
• OMIM: https://mirror.omim.org/entry/208400
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=175