Antiprion drugs are drugs that work against prions. The discovery of antiprion drugs is a priority because prion diseases are untreatable and fatal. Therefore, it is a therapeutic priority to find effective anti-prion drugs.
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. Drugs that target PrPC, the normal prion isoform, are also hypothesized to delay the progression of prion diseases.
References
- Mercer, Robert CC; Harris, David A (1 February 2019). "Identification of anti-prion drugs and targets using toxicity-based assays". Current Opinion in Pharmacology. 44: 20–27. doi:10.1016/j.coph.2018.12.005. ISSN 1471-4892. PMC 6561806. PMID 30684854.
- Bertsch, U.; Winklhofer, K. F.; Hirschberger, T.; Bieschke, J.; Weber, P.; Hartl, F. U.; Tavan, P.; Tatzelt, J.; Kretzschmar, H. A.; Giese, A. (26 May 2005). "Systematic Identification of Antiprion Drugs by High-Throughput Screening Based on Scanning for Intensely Fluorescent Targets". Journal of Virology. 79 (12): 7785–7791. doi:10.1128/JVI.79.12.7785-7791.2005. PMC 1143673. PMID 15919931.
- Le Bras, Alexandra (April 2019). "A new drug to treat prion diseases". Lab Animal. 48 (4): 116. doi:10.1038/s41684-019-0273-2. ISSN 1548-4475.