Which organelle is primarily affected in Tay-Sachs disease?

Tay-Sachs disease is primarily associated with lysosomes, which are organelles responsible for breaking down waste materials and cellular debris. In Tay-Sachs disease, there is a deficiency in an enzyme called hexosaminidase A, which is crucial for the metabolism of a specific type of fat called GM2 ganglioside within the lysosomes. When this enzyme is absent or deficient, GM2 ganglioside accumulates to toxic levels, particularly in nerve cells of the brain and spinal cord. This accumulation leads to the progressive neurodegenerative symptoms characteristic of Tay-Sachs disease. Therefore, the primary organelle affected in this condition is the lysosome due to its role in lipid metabolism and waste processing.