MeSH definition : 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.;
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.