A recessively inherited, progressive lysosomal storage disease caused by a deficiency of GLYCOSYLASPARAGINASE activity. The lack of this enzyme activity results in the accumulation of N-acetylglucosaminylasparagine (the linkage unit of asparagine-linked glycoproteins) in LYSOSOMES.

Synonyms: AGA Deficiencies, Deficiencies, AGA, Glycoasparaginase Deficiency, AGA Deficiency, Aspartylglucosaminuria, Deficiency, Aspartylglucosamidase, Aspartylglycosaminurias, Aspartylglucosamidase Deficiencies, Deficiency, Glycoasparaginase, Aspartylglycosaminuria, Deficiency, AGA, Aspartylglucosamidase Deficiency, Glycoasparaginase Deficiencies, Aspartylglucosaminurias, Deficiencies, Aspartylglucosamidase, Deficiencies, Glycoasparaginase