Loss of hexosaminidase A activity results in accumulation of

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Loss of hexosaminidase A activity results in the accumulation of G-ganglioside, a glycosphingolipid. This is the underlying cause of Tay-Sachs disease, a rare genetic disorder characterized by progressive neurological degeneration. The accumulation of G-ganglioside specifically occurs in the brain, leading to the death of neurons and the many symptoms associated with the disease. The accumulation of other lipids (such as galactocerebroside or sulfatide) can occur in other lysosomal storage disorders.

Loss of Hexosaminidase a Activity Results in Accumulation of