: a hereditary disorder of lipid metabolism typically affecting individuals of eastern European Jewish ancestry that is marked by the accumulation of lipids especially in nerve cells of the brain due to a deficiency of hexosaminidase and that is characterized by weakness, exaggerated startle response to sudden noise, diminished muscle tone, red retinal spots, macrocephaly, gradual loss of hearing and vision, deterioration of cognitive abilities and motor skills, seizures, paralysis, and death in early childhood
Tay-Sachs disease is inherited as an autosomal recessive trait requiring that both parents pass on a copy of the defective gene on a chromosome other than a sex chromosome.
… if they both had the recessive gene and ever had a baby, there was a one in four chance the baby would have Tay-Sachs disease.—Barbara Mahany In peoples around the world Tay-Sachs appears once in every 400,000 births. But it appears a hundred times more frequently—about once in 3,600 births—among descendants of Eastern European Jews, people known as Ashkenazim.—Jared Diamond
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