Molecular Pathology of the Prions

ISBN-13: 9781617372520 / Angielski / Miękka / 2010 / 279 str.

Harry F. Baker

Opis

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.

Spis treści

What Would Thomas Henry Huxley Have Made of Prion Diseases? Rosalind M. Ridley Prion Protein as Copper-Binding Protein at the Synapse Hans A. Kretzschmar, Tobias Tings, Axel Madlung, Armin Giese, and Jochen Herms A Function for the Prion Protein? David R. Brown and Ian M. Jones Prion Protein Peptide: Agents of Death for Neurons David R. Brown Characterization of Bovine Spongiform Encephalopathy and Scrapie Strains/Isolates by Immunochemical Analysis of PrPSc Martin H. Groschup, Frauke Junghans, Martin Eiden, and Thorsten Kuczius Differential Targeting of Neurons by Prion Strains Stephen J. DeArmond Transgenic Studies of Prion Diseases Glenn C. Telling Prions: From Neurografts to Neuroinvasion Markus Glatzel, Sebastian Brandner, Michael A. Klein, and Adriano Aguzzi Cellular and Transgenic Models of Familial Prion Diseases David A. Harris, Roberto Chiesa, Antonio Migheli, Pedro Piccardo, and Bernardino Ghetti Central Nervous System Inflammation and Prion Disease Pathogenesis Samar Betmouni and V. Hugh Perry The Electroneuropathology of Prion Disease J. Richard Greene Transmissible Spongiform Encephalopathy Neurobiology and Ultrastructure Suggests Extracellular PrPSc Conversion Consistent with Classical Amyloidosis Martin Jeffrey and Jan R. Fraser Conformation as Therapeutic Target in the Prionoses and Other Neurodegenerative Conditions Thomas Wisniewski, Einar M. Sigurdsson, Pierre Aucouturier, and Blas Frangione Prions of Yeast: From Cytoplasmic Genes to Heritable Amyloidosis Reed B. Wickner, Herman K. Edskes, Kimberly L. Taylor, Marie-Lise Maddelein, Hiromitsu Moriyama, and B. Tibor Roberts

Blurb

It is now widely agreed that the prion protein plays a key role in the molecular pathogenesis of prion diseases-diseases that involve the misfolding of proteins-in both humans and animals. In Molecular Pathology of the Prions, noted prion researcher Harry Baker has asked internationally recognized investigators to 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. Other topics addressed include the neuronal death that occurs in prion disease, the different strains of prion disease agents, and the accumulation of protein deposits within brain parenchyma.
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, including research techniques now likely to enjoy broader application for the more common proteinopathies, such as Alzheimer's and Parkinson's diseases.