Question

Which of the following is not a prion related disease?

Select one:

a. Phenylketonuria

b. Creutzfeldt-Jacob disease

c. Gerstmann-Straussler-Scheinker disease

d. Familial fatal insomnia

e. All of these choices are correct

Answer 1

Prion related disease are the diseases caused due to misfolding of proteins. These misfolded proteins then acts as infectious agents for the normal protein causing them to mutate. The prions aggregate in the body tissue and cause cell damage and death.

Creutzfeldt jacob disease, Gerstmann Straussler Scheinker disease and familial fatal insomnia are examples of prion diseases.

Phenylketonuria is a genetic disease. It is caused by mutation of PAH gene resulting in reduced  phenylalanine hydroxylase levels. This enzyme is responsible for metabolising amino acid phenylalanine and its low levels cause excess accumulation of phenylalanine in body.

So the answer is a) phenylketonuria.