Prion diseases recently have attracted interest not only scientifically but also socially because of the bovine spongiform encephalopathy (BSE) epidemic and the outbreak of variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom. In 2004, the International Symposium of Prion Diseases for Food and Drug Safety was held October 31–November 2 in Sendai, Japan, where, 20 years earlier, arguments were first heard on whether the etiologic agent of transmissible spongiform encephalopathy was prions or scrapie-associated fibrils. This volume is a collection of current work on prion research that was presented at the 2004 symposium. Topics included range from basic research to clinical aspects of prion diseases, making the book a valuable resource for researchers and clinicians, and encouraging further developments by the next generation of researchers.
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Table of ContentsPathology of Variant Creutzfeldt-Jakob Disease.- Clinical Aspects of Variant CJD.- Dura mater related Creutzfeldt-Jakob disease in Japan: Relationship between sites of grafts and clinical features.- Treatment Options in Patients with Prion Disease - the Role of Long Term Cerebroventricular Infusion of Pentosan Polysulphate.- Human prion diseases: novel diagnostic principles.- History and state of the art of PrP-res “typing” in Creutzfeldt-Jakob disease.- Chronic Wasting Disease in Cervids in North America.- Bovine spongiform encephalopathy (BSE) in Japan.- The role of host PrP in control of incubation time.- The role of the immune system in TSE agent neuroinvasion.- Prion protein interactions and TSE infections in cell culture models.- Semi-classical Quantization of Protein Dynamics: Novel NMR Relaxation Formalism and its Application to Prion.- Surveillance of chronic wasting disease (CWD) in Japan.- Unexpected high incidence of visceral AA-amyloidosis in slaughtered cattle in Japan.- A Novel BSE screening kit with simplified preparation method for EIA.- Detection of recombinant bovine prion protein by fluorescence correlation spectroscopy.- PrPSc distribution of a natural case of bovine spongiform encephalopathy.- Report of the first oral inoculation of BSE prion into cattle in Japan.- The development of the intracerebral inoculation method and BSE experimental transmissions to calves.- Comparative analyses of three mouse-adapted scrapie strains G1, Obihiro, and I3/I5 and pathogenesis of G1 strain-induced polyuria in ICR mice.- Dose effect on detection of PrPSc in follicular dendritic cells of knock-in mice for rapid bioassay.- Cell surface retention of PrPC by anti-PrP antibody prevents protease-resistant PrP formation.- Prion-conformation-specific human antibodies established from phage display library.- Partial characterization of monoclonal antibodies which bind to disease-associated prion protein in immunoprecipitaion assay.- Production and Characterization of Monoclonal Antibodies Specific for Prion Protein.- In vitro selection of anti-mouse prion protein RNA aptamers.- Detection of the Prion Protein in a Liquid Phase Capture Assay Using Magnetic Beads Coupled to Protein A.- New pretreatment method for immunohistochemistry for abnormal prion protein.- Cellular prion protein suppresses the apoptosis in a neuronal cell line established from type-1 prion protein gene-deficient mice.- Cellular prion protein suppressess the apoptotic cell death by mediating the intracellular H2O2 in primary culture and immortalized neuronal cells.- Targeting of cytosolic PrPC via a novel 14-3-3-Tom 70-mitochondrial BCL-2 pathway induces mitochondrial apoptosis.- Microtubule-dependent intracellular trafficking of cellular prion protein.- Hsp90 modifies the conformation of recombinant mouse prion protein in vitro.- Purification and characterization of a novel ATP-dependent robust protein-unfoldase, Unfoldin.- Nucleic acid and Prion protein interaction produces spherical amyloids which in vivo can function as coats of Spongiform Encephalopathy agent.- Species barrier in yeast [PSI+] prion transmission.- Molecular memory of [PSI+] prion strains in S. cerevisiae is dependent on the primary structure of the Sup35 prion domain.- Expression of a splice variant of prion protein during hypoxia in human glioblastoma cell line T98G.- PRNP promoter region polymorphism in Creutzfeldt-Jakob disease.- Tubulovesicular structures are consistently found in prion diseases including vCJD and FFI.- Autophagy is a common ultrastructural feature of neuropathology of prion diseases.- Type 1 and type 2 human PrPSc have different aggregation sizes in methionine homozygotes with sporadic, iatrogenic and variant Creutzfeldt-Jakob disease.- A pitfall in diagnosis of human prion diseases using detection of protease-resistant prion protein in urine: contamination with bacterial outer membrane proteins.- Comparative analysis of chronological data of total tau protein in CSF, serial studies of MRI (DWI, and FLAIR) and staging of clinical features in patients with sporadic CJD.- Duration between initial manifestation of CJD and detection of PSD, specific findings on MRI, CSF 14-3-3 protein, or CSF high NSE.- Biochemical analysis of peripheral tissue involvement in transmissible spongiform encephalopathies.- Involvement of the peripheral nervous system in human prion diseases including dural graft-associated Creutzfeldt-Jakob disease.- MM2 type sporadic Creutzfeldt-Jakob disease: clinicoradiologic features and clinical diagnosis.- Early clinical and radiological diagnosis of sporadic Creuzfeldt-Jakob’s disease (sCJD) a case study of pathologically-proved sCJD MV2 .- Familial Creutzfeldt-Jakob disease with a point mutation (Met to Arg) at codon 232: two different phenotypes.- Familial Creutzfeldt-Jakob disease with five octapeptide repeat insert.- A case of Creutzfeldt-Jakob disease with a novel insertion mutation and codon 219 Lysine/Lysine polymorphism in the prion protein gene.- Inhibition of Prion Propagation in Scrapie-infected Cell Lines using Mouse Monoclonal Antibodies against Prion Protein.- The inhibitory effect of the ScFv of an anti-prion protein antibody secreted from N2a58 cells on abnormal prion protein accumulation in scrapie-infected cells, ScN2a.- Mucosal immunogenicity of prion protein fused with heat-labile enterotoxin B subunit.- Identification of drugs that enhance the stimulatory effect of PrP on the fibrinolytic system.- Screening study of prion binding agents and their inhibitory effect on the conversion of prion protein.- Can Forage Grasses inhibit Prion Replication?.- Inhibition of abnormal PrP formation by amyloid-imaging probes in vitro.- Treatment with Anti-malaria Agents, Quinacrine and Quinine, for Creutzfeldt - Jakob disease patients.- Effect of oral administration of pentosan polysulfate for patients with Creutzfeldt-Jakob disease (CJD) and new design for low molecular-weight of pentosan polysulfate.