PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 27906067-4 2016 Progressive intellectual and physical disability is the main symptom leading to death usually before the age of 50 years.The disease is caused by the deficient activity of the lysosomal enzyme glycosylasparaginase (aspartylglucosaminidase, AGA), which leads to a disorder in the degradation of glycoasparagines - aspartylglucosamine or other glycoconjugates with an aspartylglucosamine moiety at their reducing end - and accumulation of these undegraded glycoasparagines in tissues and body fluids. Glycoconjugates 342-357 aspartylglucosaminidase Homo sapiens 240-243