Hereditary prion protein amyloidoses.

  title={Hereditary prion protein amyloidoses.},
  author={Bernardino Ghetti and Fabrizio Tagliavini and Masashi Takao and Orso Bugiani and Pedro Piccardo},
  journal={Clinics in laboratory medicine},
  volume={23 1},
  pages={65-85, viii}
Prion protein (PrP) amyloid accumulation is the pathologic hallmark of some inherited prion diseases such as Gerstmann-Sträussler-Scheinker disease (GSS) and PrP cerebral amyloid angiopathy (PrP-CAA). In GSS, parenchymal amyloidosis may coexist with spongiform degeneration or neurofibrillary tangles, whereas in PrP-CAA, vascular amyloid coexists with neurofibrillary tangles. In GSS, N-truncated and C-truncated proteinase K-resistant PrP isoforms are present in the brain.