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 __________ _________ ____ _______ __________ ___ __________ ______.
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