Name the defective enzyme in the following diseases:

a) Tyrosinemia

b) Phenylketonurea

c) Alkaptonurea

d) Niemann-Pick-Disease

e) Von-Gierke's Syndrome

a) Tyrosinemia: Tyrosinemia is caused by a defect in the enzyme fumarylacetoacetate hydrolase. This enzyme is responsible for the final step in the degradation of the amino acid tyrosine. The defective enzyme leads to the accumulation of toxic metabolites, causing liver and kidney damage.

b) Phenylketonuria (PKU): In PKU, the defective enzyme is phenylalanine hydroxylase. ________ ____ _______ ___ __________ ______ ____ _________ ____ ________.
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