Answered>Order 9101
Children with I-cell Disease synthesize perfectly good lysosomal enzymes, but secrete them outside the cell instead of sorting them to lysosomes. The mistake occurs because the cells lack GlcNac-P transferase, which is required to create the mannose-6-phosphate marker that is essential for proper delivery of hydrolytic enzymes into the lysosomes. In principle, I-cell disease could […]