Tags: Disease.
Sandhoff disease also known as Sandhoff-Jatzkewitz disease variant 0 of GM2-Gangliosidosis or Hexosaminidase A and B deficiency 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.