The cellular accumulation seen in Tay-Sachs disease is primarily within which organelle?

The cellular accumulation seen in Tay-Sachs disease is primarily within which organelle?

• A. Lysosome
• B. Nucleus
• C. Rough ER
• D. Golgi

Answer: (A)

Tay-Sachs disease is a lysosomal storage disorder, so the buildup happens inside lysosomes. The defect is in beta-hexosaminidase A, an enzyme that degrades GM2 ganglioside. When this enzyme is missing, GM2 cannot be broken down and accumulates within lysosomes, particularly in neurons, leading to progressive neurodegeneration. Other organelles like the nucleus, rough ER, or Golgi are not the sites where this lipid storage occurs; they’re involved in genetic information, protein synthesis, and trafficking, respectively, not in the degradation of GM2. Therefore, the organelle primarily involved is the lysosome.

Tay-Sachs disease is a lysosomal storage disorder, so the buildup happens inside lysosomes. The defect is in beta-hexosaminidase A, an enzyme that degrades GM2 ganglioside. When this enzyme is missing, GM2 cannot be broken down and accumulates within lysosomes, particularly in neurons, leading to progressive neurodegeneration. Other organelles like the nucleus, rough ER, or Golgi are not the sites where this lipid storage occurs; they’re involved in genetic information, protein synthesis, and trafficking, respectively, not in the degradation of GM2. Therefore, the organelle primarily involved is the lysosome.