Enzyme deficient in Tay Sach disease:

Tay-Sachs diseaseIt is caused by insufficient activity of the enzyme hexosaminidase A.Hexosaminidase A is a vital hydrolytic enzyme, found in the lysosomes, that breaks down glycolipids.When hexosaminidase A is no longer functioning properly, the lipids accumulate in the brain and interfere with normal biological processes.Hexosaminidase A specifically breaks down fatty acid derivatives called gangliosides; these are made and biodegraded rapidly in early life as the brain develops.