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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.
Children with Tay-Sachs disease lack a vital enzyme, hexosaminidase A (Hex-A). Hex-A is needed for the body to break down a fatty waste substance found in brain cells.Without Hex-A, this substance accumulates abnormally and causes progressive damage until the nervous system can no longer sustain life.hen both parents are carriers, there is a 2 in 4 (50%) chance, with every pregnancy, of having a child who is a carrier. When only one parent is a carrier, there is no chance the child will have Tay-Sachs disease.