Internationally recognized investigators review the latest developments in, and novel approaches to, understanding the prion protein and prion diseases at the molecular level. Utilizing a variety of cutting-edge techniques, these distinguished scientists seek to define the normal function of a prion protein, to detect and measure the early immune response to prion disease, and to discover possible therapeutic targets. They also use transgenic mice and new electrophysiological investigations to elucidate the pathogenetic mechanisms involved in prion diseases. State-of-the-art and richly insightful, Molecular Pathology of the Prions captures for basic and clinical neuropathologists the latest developments and approaches to understanding the pathogenesis of prion diseases, and by analogy suggests possible research techniques for the more common proteinopthies, such as Alzheimer's and Parkinson's diseases.

Includes supplementary material: sn.pub/extras

What Would Thomas Henry Huxley Have Made of Prion Diseases?Rosalind M. RidleyPrion Protein as Copper-Binding Protein at the SynapseHans A. Kretzschmar, Tobias Tings, Axel Madlung, Armin Giese, and Jochen HermsA Function for the Prion Protein?David R. Brown and Ian M. JonesPrion Protein Peptide: Agents of Death for NeuronsDavid R. BrownCharacterization of Bovine Spongiform Encephalopathy and Scrapie Strains/Isolates by Immunochemical Analysis of PrPScMartin H. Groschup, Frauke Junghans, Martin Eiden, and Thorsten KucziusDifferential Targeting of Neurons by Prion StrainsStephen J. DeArmondTransgenic Studies of Prion DiseasesGlenn C. TellingPrions: From Neurografts to NeuroinvasionMarkus Glatzel, Sebastian Brandner, Michael A. Klein, and Adriano AguzziCellular and Transgenic Models of Familial Prion DiseasesDavid A. Harris, Roberto Chiesa, Antonio Migheli, Pedro Piccardo, and Bernardino GhettiCentral Nervous System Inflammation and Prion Disease PathogenesisSamar Betmouni and V. Hugh PerryThe Electroneuropathology of Prion DiseaseJ. Richard GreeneTransmissible Spongiform Encephalopathy Neurobiology and Ultrastructure Suggests Extracellular PrPSc Conversion Consistent with Classical AmyloidosisMartin Jeffrey and Jan R. FraserConformation as Therapeutic Target in the Prionoses and Other Neurodegenerative ConditionsThomas Wisniewski, Einar M. Sigurdsson, Pierre Aucouturier, and Blas FrangionePrions of Yeast: From Cytoplasmic Genes to Heritable AmyloidosisReed B. Wickner, Herman K. Edskes, Kimberly L. Taylor, Marie-Lise Maddelein, Hiromitsu Moriyama, and B. Tibor Roberts