<prompt> Hexosaminidase A deficiency causes:Niemann-pickTay-Sachs diseaseHurler syndromeGauchers disease  <response>: Ans: b. Tay-Sachs disease Refs Harper 30th251; Lippincott 6th212; Harrison 19th432e,433eHexosaminidase A deficiency is an enzyme deficiency that causes brain and other nerve cells to die, which can lead to severe neurological and mental problems.Hexosaminidase A HEX A deficiency is caused by a deficiency in an enzyme called beta-hexosaminidase A. This enzyme helps break down a paicular fatty acid called GM2 ganglioside.Without adequate amounts of functional enzymes, GM2 ganglioside will build up in nerve cells and cause them to die.There are several forms of HEX A deficiency, including acute infantile Tay-Sachs disease, juvenile, chronic, or adult-onset forms. 