Loading Pathway...
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
Nucleus Lysosome AGA AGA Aspartylglycosamine N-Acetyl-b- glucosaminylamine L-Aspartic acid Glycoprotein AGA