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Tay-Sachs disease


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(amaurotic familial idiocy) an inherited disorder of lipid metabolism (see lipidosis) in which abnormal accumulation of lipid in the brain leads to blindness, mental retardation, and death in infancy. The gene responsible for the disorder is recessive, and the disease can now be largely prevented by genetic counselling in communities known to be affected (primarily Ashkenazi Jewish communities). W. Tay (1843–1927), British physician; B. Sachs (1858–1944), US neurologist


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