Aspartylglucosaminidase is involved in the catabolism of N-linked oligosaccharides of glycoproteins. 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. Alternatively spliced transcript variants have been identified.
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Protein Aliases: AGA; Aspartylglucosaminidase; aspartylglucosylamine deaspartylase; Glycosylasparaginase; Glycosylasparaginase alpha chain; Glycosylasparaginase beta chain; N(4)-(Beta-N-acetylglucosaminyl)-L-asparaginase; N4-(N-acetyl-beta-glucosaminyl)-L-asparagine amidase
Gene Aliases: AGA; AGU; ASRG; AW060726; GA