In a lysosomal storage disease,newly synthesized lysosome hydrolases are secreted from the cells rather than being delivered to the lysosomes.What is one possible mechanism for this result?
A)The cis-Golgi network lacks a proton gradient.
B)The ER is unable to produce lysosomal vesicles.
C)Lysosomal enzymes are not phosphorylated in the rER.
D)Mannose is not phosphorylated in the cis-Golgi network.
E)All the hydrolase genes are mutated,resulting in synthesis by ribosomes free in the cytoplasm rather than on the endoplasmic reticulum membrane.

Question

Quizplus · Accepted Answer

Mannose-6-phosphate is a crucial signal for targeting lysosomal enzymes to lysosomes. If mannose is not phosphorylated in the cis-Golgi network, the enzymes are not properly tagged for lysosomal delivery and are instead secreted outside the cell.

In a Lysosomal Storage Disease,newly Synthesized Lysosome Hydrolases Are Secreted