Which of the following occurs in the lipidosis known as Tay-Sachs disease?

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In Tay-Sachs disease, there is a deficiency of the enzyme hexosaminidase A, leading to the accumulation of ganglioside GM2 in the lysosomes, as it is not properly catabolized. In the genetic disorder known as TaySachs disease, ganglioside G_M2 is not catabolized. As a consequence, the ganglioside concentration is elevated many times higher than normal. The functionally absent lysosomal enzyme is β-N-acetylhexosaminidase (more commonly called hexosaminidase A). The elevated G_M2 results in irreversible brain damage to infants, who usually die before the age of 3 years. Under normal conditions, this enzyme cleaves N-acetylgalactosamine from the oligosaccharide chain of this complex sphingolipid, allowing further catabolism to occur.

Which of the Following Occurs in the Lipidosis Known as Tay-Sachs