Autosomal inherited disease caused by deficiency of the enzymes hexosaminidase a and b which leads to an accumulation of gm2 ganglioside and the sphingolipid globoside in neurons and other organs; clinical manifestations resemble tay-sachs disease.

Sandhoff disease is a lysosomal genetic, lipid storage disorder caused by the inherited deficiency to create functional beta-hexosaminidases A and B. These catabolic enzymes are needed to degrade the neuronal membrane components, ganglioside GM2, its derivative GA2, the glycolipid globoside in visceral tissues, and some oligosaccharides...