Definition of Hexosaminidase AHexosaminidase A: An enzyme which, when deficient, causes Tay-Sachs disease, a progressive fatal neurologic disorder concentrated in people of European Jewish (Ashkenazi) descent. See: Tay-Sachs disease. Source: MedTerms™ Medical Dictionaryhttp://www.medterms.com/script/main/art.asp?articlekey=3749 Last Editorial Review: 10/9/2012
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