Prusiner, S. B. Prions. Proc. Natl Acad. Sci. USA 95, 13363–13383 (1998).

CAS  Article  Google Scholar 

Cobb, N. J. & Surewicz, W. K. Prion diseases and their biochemical mechanisms. Biochemistry 48, 2574–2585 (2009).

CAS  Article  Google Scholar 

Caughey, B. & Kraus, A. Transmissibility versus pathogenicity of self-propagating protein aggregates. Viruses 11, 1044 (2019).

CAS  Article  Google Scholar 

Kitamoto, T., Iizuka, R. & Tateishi, J. An amber mutation of prion protein in Gerstmann–Sträussler syndrome with mutant PrP plaques. Biochem. Biophys. Res. Commun. 192, 525–531 (1993).

CAS  Article  Google Scholar 

Ghetti, B. et al. Vascular variant of prion protein cerebral amyloidosis with τ-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP. Proc. Natl Acad. Sci. USA 93, 744–748 (1996).

CAS  Article  Google Scholar 

Choi, J. K. et al. Amyloid fibrils from the N-terminal prion protein fragment are infectious. Proc. Natl Acad. Sci. USA 113, 13851–13856 (2016).

CAS  Article  Google Scholar 

Vanik, D. L., Surewicz, K. A. & Surewicz, W. K. Molecular basis of barriers for interspecies transmissibility of mammalian prions. Mol. Cell 14, 139–145 (2004).

CAS  Article  Google Scholar 

Jones, E. M. & Surewicz, W. K. Fibril conformation as the basis of species- and strain-dependent seeding specificity of mammalian prion amyloids. Cell 121, 63–72 (2005).

CAS  Article  Google Scholar 

Theint, T. et al. Species-dependent structural polymorphism of Y145Stop prion protein amyloid revealed by solid-state NMR spectroscopy. Nat. Commun. 8, 753 (2017).

Article  Google Scholar 

Cao, Q., Boyer, D. R., Sawaya, M. R., Ge, P. & Eisenberg, D. S. Cryo-EM structures of four polymorphic TDP-43 amyloid cores. Nat. Struct. Mol. Biol. 26, 619–627 (2019).

CAS  Article  Google Scholar 

Bansal, A. et al. AA amyloid fibrils from diseased tissue are structurally different from in vitro formed SAA fibrils. Nat. Commun. 12, 1013 (2021).

CAS  Article  Google Scholar 

Theint, T. et al. Structural studies of amyloid fibrils by paramagnetic solid-state nuclear magnetic resonance spectroscopy. J. Am. Chem. Soc. 140, 13161–13166 (2018).

CAS  Article  Google Scholar 

Kundu, B. et al. Nucleation-dependent conformational conversion of the Y145Stop variant of human prion protein: structural clues for prion propagation. Proc. Natl Acad. Sci. USA 100, 12069–12074 (2003).

CAS  Article  Google Scholar 

Collinge, J. & Clarke, A. R. A general model of prion strains and their pathogenicity. Science 318, 930–936 (2007).

CAS  Article  Google Scholar 

Apostol, M. I., Wiltzius, J. J. W., Sawaya, M. R., Cascio, D. & Eisenberg, D. Atomic structures suggest determinants of transmission barriers in mammalian prion disease. Biochemistry 50, 2456–2463 (2011).

CAS  Article  Google Scholar 

Wang, L. Q. et al. Cryo-EM structure of an amyloid fibril formed by full-length human prion protein. Nat. Struct. Mol. Biol. 27, 598–602 (2020).

Article  Google Scholar 

Wang, L. et al. Genetic prion disease-related mutation E196K displays a novel amyloid fibril structure revealed by cryo-EM. Sci. Adv. 7, eabg9676 (2021).

CAS  Article  Google Scholar 

Kraus, A. et al. High-resolution structure and strain comparison of infectious mammalian prions. Mol. Cell 81, 4540–4551.e6 (2021).

CAS  Article  Google Scholar 

Artikis, E., Kraus, A. & Caughey, B. Structural biology of ex vivo mammalian prions. J. Biol. Chem. 298, 102181 (2022).

CAS  Article  Google Scholar 

Glynn, C. et al. Cryo-EM structure of a human prion fibril with a hydrophobic, protease-resistant core. Nat. Struct. Mol. Biol. 27, 417–423 (2020).

CAS  Article  Google Scholar 

Kyte, J. & Doolittle, R. F. A simple method for displaying the hydropathic character of a protein. J. Mol. Biol. 157, 105–132 (1982).

CAS  Article  Google Scholar 

Morillas, M., Swietnicki, W., Gambetti, P. & Surewicz, W. K. Membrane environment alters the conformational structure of the recombinant human prion protein. J. Biol. Chem. 274, 36859–36865 (1999).

CAS  Article  Google Scholar 

Yu, G., Li, K. & Jiang, W. Antibody-based affinity cryo-EM grid. Methods 100, 16–24 (2016).

CAS  Article  Google Scholar 

Bokori-Brown, M. et al. Cryo-EM structure of lysenin pore elucidates membrane insertion by an aerolysin family protein. Nat. Commun. 7, 11293 (2016).

CAS  Article  Google Scholar 

Mastronarde, D. N. Automated electron microscope tomography using robust prediction of specimen movements. J. Struct. Biol. 152, 36–51 (2005).

Article  Google Scholar 

Zheng, S. Q. et al. MotionCor2: anisotropic correction of beam-induced motion for improved cryo-electron microscopy. Nat. Methods 14, 331–332 (2017).

CAS  Article  Google Scholar 

Rohou, A. & Grigorieff, N. CTFFIND4: fast and accurate defocus estimation from electron micrographs. J. Struct. Biol. 192, 216–221 (2015).

Article  Google Scholar 

Scheres, S. H. W. RELION: implementation of a Bayesian approach to cryo-EM structure determination. J. Struct. Biol. 180, 519–530 (2012).

CAS  Article  Google Scholar 

He, S. & Scheres, S. H. W. Helical reconstruction in RELION. J. Struct. Biol. 198, 163–176 (2017).

CAS  Article  Google Scholar 

Zivanov, J., Nakane, T. & Scheres, S. H. W. A Bayesian approach to beam-induced motion correction in cryo-EM single-particle analysis. IUCrJ 6, 5–17 (2019).

CAS  Article  Google Scholar 

Zivanov, J., Nakane, T. & Scheres, S. H. W. Estimation of high-order aberrations and anisotropic magnification from cryo-EM data sets in RELION-3.1. IUCrJ 7, 253–267 (2020).

CAS  Article  Google Scholar 

Emsley, P., Lohkamp, B., Scott, W. G. & Cowtan, K. Features and development of Coot. Acta Crystallogr. D Biol. Crystallogr. 66, 486–501 (2010).

CAS  Article  Google Scholar 

Afonine, P. V. et al. Real-space refinement in PHENIX for cryo-EM and crystallography. Acta Crystallogr. D Struct. Biol. 74, 531–544 (2018).

CAS  Article  Google Scholar 

Liebschner, D. et al. Macromolecular structure determination using X-rays, neutrons and electrons: recent developments in Phenix. Acta Crystallogr. D Struct. Biol. 75, 861–877 (2019).

CAS  Article  Google Scholar 

Afonine, P. V. et al. New tools for the analysis and validation of cryo-EM maps and atomic models. Acta Crystallogr. D Struct. Biol. 74, 814–840 (2018).

CAS  Article  Google Scholar 

Pettersen, E. F. et al. UCSF Chimera—a visualization system for exploratory research and analysis. J. Comput. Chem. 25, 1605–1612 (2004).

CAS  Article  Google Scholar