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Related to Tay-Sachs disease: phenylketonuria, Niemann-Pick disease, cystic fibrosis, sickle cell anemia
A hereditary disease that affects young children almost exclusively of eastern European Jewish descent, in which an enzyme deficiency leads to the accumulation of gangliosides in the brain and nerve tissue, resulting in intellectual disability, convulsions, blindness, and, ultimately, death.
[After Warren Tay (1843-1927), British physician, and Bernard, Sachs (1858-1944), American neurologist.]
American Heritage® Dictionary of the English Language, Fifth Edition. Copyright © 2016 by Houghton Mifflin Harcourt Publishing Company. Published by Houghton Mifflin Harcourt Publishing Company. All rights reserved.
(Pathology) an inherited disorder, caused by a faulty recessive gene, in which lipids accumulate in the brain, leading to mental retardation and blindness. It occurs mostly in Ashkenazi Jews
[C20: named after W. Tay (1843–1927), British physician, and B. Sachs (1858–1944), US neurologist]
Collins English Dictionary – Complete and Unabridged, 12th Edition 2014 © HarperCollins Publishers 1991, 1994, 1998, 2000, 2003, 2006, 2007, 2009, 2011, 2014
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.
[1905–10; after Warren Tay (1843–1927), British ophthalmologist, and Bernard Sachs (1858–1944), U.S. neurologist, who described it independently]
Random House Kernerman Webster's College Dictionary, © 2010 K Dictionaries Ltd. Copyright 2005, 1997, 1991 by Random House, Inc. All rights reserved.
A hereditary disease in which the products of fat metabolism accumulate in the nervous system, causing retardation, paralysis, and death by the age of 3 or 4. It mostly affects children of eastern European Jewish descent.
The American Heritage® Student Science Dictionary, Second Edition. Copyright © 2014 by Houghton Mifflin Harcourt Publishing Company. Published by Houghton Mifflin Harcourt Publishing Company. All rights reserved.
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|Noun||1.||Tay-Sachs disease - 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|
autosomal recessive defect, autosomal recessive disease - a disease caused by the presence of two recessive mutant genes on an autosome
lipidosis - a disorder of lipid metabolism; abnormal levels of certain fats accumulate in the body
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