Aspartylglycosaminuria (AGU) is an autosomal recessive lysosomal storage disease belonging to the oligosaccharidosis group (also called glycoproteinosis). The disease is Monarch Disease Ontology identifier MONDO_0008830 (aspartylglucosaminuria). Also known as: Aspartylglycosaminuria, aspartylglucosaminidase deficiency, aspartylglycosaminuria, glycosylasparaginase deficiency.