<unk>  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. Accumulation of these metabolites leads to a progressive destruction of the central nervous system and eventually to death. The rare autosomal recessive neurodegenerative disorder is clinically almost indistinguishable from Tay–Sachs disease, another genetic disorder that disrupts beta-hexosaminidases A and S. There are three subsets of<unk>  based on when first symptoms appear: classic infantile, juvenile and adult late onset.
Sandhoff disease
Tay–Sachs disease
Stratton Parker syndrome
Menkes disease
Gerstmann–Sträussler–Scheinker syndrome
Scheie syndrome
Aceruloplasminemia
Batten disease