N-terminal sequence of prion protein is also integrated into kuru plaques in patients with Gerstmann-Sträussler syndrome.

Kuru plaques are one of the pathological hallmarks in Gerstmann-Sträussler syndrome, and are composed of prion protein (PrP). To elucidate whether N-terminal sequence of PrP is related to amyloid formation in vivo, we prepared antibody against synthetic N-terminal peptide (anti-PrP-N). Anti-PrP-N immunolabeled kuru plaques positively. Positive reactions were observed in the periphery of large kuru plaque cores, but not in the center. It is therefore postulated that one of the modifications of PrP is N-terminal truncation.