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Tay-Sachs disease An inherited condition characterized by the absence of a single lysosomal enzyme called Hex A, which normally breaks down a membrane glycolipid called ganglioside G_M2 that is especially prevalent in nerve cells; usually occurs in children of Ashkenazi (eastern European Jewish) descent and is characterized by seizures and muscle rigidity.
Tay-Sachs is a disease that is the result of a genetic mutation; it is fatal and affects the central nervous system. It is the result of the HEXA gene, located on chromosome 15; what occurs is that children have the deficiency of the enzyme, Hex X.here are two different kinds of this; infantile tay-sachs disease and late onset of tay-sachs disease. Infantile Tay-Sachs starts to show signs when the infant is around six months old; by age two, most children with this condition have seizures and decreasing mental state.