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1. (n.) Tay-Sachs disease
a degenerative brain disorder caused by lack of or deficiency in an essential enzyme, usu. resulting in mental and physical deterioration and death in early childhood.
Etymology: (1905–10; after Warren Tay (1843–1927), British ophthalmologist, and Bernard Sachs (1858–1944), U.S. neurologist, who described it independently)
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| Definition of 'tay-sachs disease' |
Princeton's WordNet |
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1. (noun) Tay-Sachs disease, Tay-Sachs, Sachs disease, infantile amaurotic idiocy
a hereditary disorder of lipid metabolism occurring most frequently in individuals of Jewish descent in eastern Europe; accumulation of lipids in nervous tissue results in death in early childhood
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| Definition of 'tay-sachs disease' |
U.S. National Library of Medicine |
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1. tay-sachs disease
An autosomal recessive neurodegenerative disorder characterized by the onset in infancy of an exaggerated startle response, followed by paralysis, dementia, and blindness. It is caused by mutation in the alpha subunit of the HEXOSAMINIDASE A resulting in lipid-laden ganglion cells. It is also known as the B variant (with increased HEXOSAMINIDASE B but absence of hexosaminidase A) and is strongly associated with Ashkenazic Jewish ancestry.
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