This technology provides a pharmaceutical composition for the prevention and treatment of prion diseases, a health functional food for prevention and mitigation, and a feed composition for livestock, all containing furanodienone derived from the medicinal herb Curcuma zedoaria. Furanodienone prevents the misfolding and aggregation of normal prion proteins, thereby inhibiting the increase of pathogenic scrapie prions.
Prion diseases are fatal conditions where misfolded scrapie prion proteins form aggregates that kill neurons and cause the brain to degenerate into a sponge-like state. Examples include bovine spongiform encephalopathy and Creutzfeldt-Jakob disease in humans, with links also reported to Alzheimer's disease. However, while quinacrine showed promise in vitro, it proved ineffective in patients and caused liver failure, and pentosan polysulfate has shown only limited efficacy, leaving a lack of viable treatment options.
This technology leverages the fact that furanodienone, a component of Curcuma zedoaria traditionally used to treat blood stasis, reduces intracellular scrapie prion levels and inhibits protein aggregation. It targets Creutzfeldt-Jakob disease, fatal familial insomnia, Gerstmann-Sträussler-Scheinker syndrome, and Alzheimer's disease, as well as scrapie in sheep and goats, and chronic wasting disease in deer, elk, and reindeer. Applicable to human prion disease and protein-aggregation dementia treatments, livestock feed for disease prevention, and neuroprotective health supplements, it offers a path to block prion spread in both humans and animals using a plant-derived compound, overcoming the efficacy and toxicity issues of previous candidates.
This invention was developed with support from the Ministry of Science and ICT for research on treating prion diseases through the enhancement of plasmin activity.
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