Which of the following is a GM2 gangliosidosis?: (A) Sandhoff's disease, (B) Niemann-Pick disease, (C) Gaucher's disease, (D) Fabry's disease
Answer is A. Sandhoff's disease and Tay-Sachs disease are GM2 gangliosidosis. GM2 Gangliosidoses Disease Deficient enzyme Lipid accumulating Clinical features Tay-Sachs disease Hexosaminidase A GM2 ganglioside Mental retardation, Blindness, Muscular weakness, Cherry-red spot Sandhoff disease b-Hexosaminidases A and B Macrocephaly Hyperacusis Cherry-red spot