A FRET-BASED METHOD TO MEASURE SEEDING ACTIVITY OF MISFOLDED PROTEIN SPECIES IN BIOLOGICAL SAMPLES

摘要

The present invention relates to a method for the quantification of seeding (Δt₅₀) of an amyloidogenic aggregate, methods for assessing the risk for development, predicting the onset or assessing the progression of a polyQ disease, and a method for identifying compounds that inhibit mHTT seeding activity (HSA) in vitro. Further, uses of fluorophore-bearing polyQ proteins, particularly mutant N-terminal huntingtin fragments comprising exon-1 and related soluble protein constructs are provided.