N(4)-(beta-N-
acetylglucosaminyl)-
L- asparaginase
N(4)-(beta-N-
acetylglucosaminyl)-
L- asparaginase
Aspartylglycosamine
N-Acetyl-b-
glucosaminylamine
L-Aspartic acid
Glycoprotein
AGA
mutation
Accumulation of undegraded
glycosidases (e.g.
Glycoprotein,
aspartylglycosamine) will
cause the deficient of
lysosome