The genetic forms of prion diseases result from mutations in the prion gene.What is the proposed mechanism for how these mutations cause the prion disease?

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The proposed mechanism for how mutations in the prion gene cause prion disease is that they cause the prion protein to misfold more easily into the PrPˢᶜ form, which is the disease-causing form. This misfolded form then aggregates and accumulates in the brain, leading to neurodegeneration and other symptoms of the disease.

The Genetic Forms of Prion Diseases Result from Mutations in the Prion