Antiprion drugs are drugs that work against prions. The discovery of antiprion drugs is a priority because prion diseases are untreatable and fatal. ^[1] Therefore, it is a therapeutic priority to find effective anti-prion drugs.^{[ citation needed]}

Mechanism of action

The disease progression in prion diseases is probably due to the conformational change of the prion protein (PrP). PrP changes from alpha-helical conformation to a disease-associated, mainly beta-sheeted scrapie isoform (PrPSc), which forms amyloid aggregates. The drugs that contain N′-benzylidene-benzohydrazide core structure are likely to slow down this progression. ^[2] Drugs that target PrPC, the normal prion isoform, are also hypothesized to delay the progression of prion diseases. ^[3]

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