Question

Lysosomes digest food particles brought into a cell by endocytosis. After a vesicle containing food particles fuses with a lysosome, H+ ions are transported into the lysosome from the cytosol. This significantly lowers the pH of the lysosome relative to the cytosol and activates the enzymes that digest the particles.
Which of the following best predicts what will happen to the lysosomal enzymes if the proteins that transport H+ ions from the cytosol into the lysosome are damaged?

Answer 1

Lysosomes are acidic compartments filled with more than 60 different types of hydrolases. They mediate the degradation of extracellular particles from endocytosis and of intracellular components from autophagy. The digested products are transported out of the lysosome via specific catabolite exporters or via vesicular membrane trafficking. Lysosomes also contain more than 50 membrane proteins and are equipped with the machinery to sense nutrient availability, which determines the distribution, number, size, and activity of lysosomes to control the specificity of cargo flux and timing (the initiation and termination) of degradation. Defects in degradation, export, or trafficking result in lysosomal dysfunction and lysosomal storage diseases (LSDs). Lysosomal channels and transporters mediate ion flux across perimeter membranes to regulate lysosomal ion homeostasis, membrane potential, catabolite export, membrane trafficking, and nutrient sensing. Dysregulation of lysosomal channels underlies the pathogenesis of many LSDs and possibly that of metabolic and common neurodegenerative diseases




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