Prions and Prion Diseases : New Developments.
Jean-Michel. Verdier
Bok Engelsk 2012 · Electronic books.
| Omfang | 1 online resource (212 pages)
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| Utgave | 1st ed.
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| Opplysninger | Intro -- PRIONS AND PRION DISEASES -- PRIONS AND PRION DISEASES -- CONTENTS -- PREFACE -- NEUROPATHOLOGY OF HUMAN PRION DISEASES -- ABSTRACT -- INTRODUCTION -- THE ROLE OF NEUROPATHOLOGY -- PERFORMING THE AUTOPSY -- NEUROPATHOLOGICAL EXAMINATION -- A.) Macroscopy -- B.) Work-Up for Histological Evaluation -- C.) Microscopic Assessment -- I.) Spongiform Change of the Neuropil -- a) In Sporadic Forms -- b.) In Genetic Forms -- c.) In Acquired Forms -- II) Neuronal Death -- a.) In Sporadic Forms -- b.) In Genetic Forms -- c.) In Acquired Forms -- SYNAPTIC PATHOLOGY -- III.) Astroglial Proliferation -- IV.) Microglial Activation -- V.) PrP Amyloid Plaques -- a.) In Sporadic Forms -- b.) In Genetic Forms -- c.) In Acquired Forms -- VI.) Cerebellar Pathology -- a.) In Sporadic Forms -- b.) In Genetic Forms -- c.) In Acquired Forms -- RELATIVE RESISTANCE OF HIPPOCAMPUS IN CJD -- VII.) Detection of PrP -- VIIa.) By Immunohistochemistry -- a.) In Sporadic Forms -- b.) In Genetic Forms -- c.) In Acquired Forms -- VIIb.) By histoblot and Paraffin-Embedded Tissue Blot -- VIId.) By Western blot -- a.) In Sporadic Forms -- b.) In Genetic Forms -- c.) In Acquired Forms -- VIII.) Co-deposition of other Abnormal Protein Aggregates in CJD -- a) In Sporadic Forms -- b) In Genetic Forms -- c) In Acquired Forms -- IX.) Differential Diagnoses from Autopsy Series -- X.) Neuronal Systems and Their Clinico-Pathological Correlates -- CONCLUSION -- ACKNOWLEDGMENTS -- REFERENCES -- THE NATURE OF THE INFECTIOUS AGENTS: PRP MODELS OF RESISTANT SPECIES TO PRION DISEASES (DOG, RABBIT AND HORSES) -- Abstract -- 1.Introduction -- 2.MaterialsandMethods -- 3.ResultsandDiscussion -- 4.Conclusion -- Acknowledgments -- References -- INDUCING TRANSMISSIBLE PRION DISEASES WITH RECOMBINANT FIBRILS: A NEW CONCEPT ON GENESIS AND EVOLUTION OF INFECTIOUS PRIONS -- ABSTRACT.. - ANTI-PRION STRATEGIES FOR IN VIVO GENE THERAPY ASSAYS -- ABSTRACT -- INTRODUCTION -- DEPLETING PRPC WITH SIRNA: AN APPROACH TO REMOVE THE KEY SUBSTRATE ESSENTIAL FOR CONVERSION AND FORMATION OF NEUROTOXIC PRION SPECIES -- PRION ANTAGONISTS WITH DOMINANT NEGATIVE EFFECT TO INHIBIT CONVERSION PROCESS: NATURAL POLYMORPHYSM PRPQ167R AND SOLUBLE DIMERIC PRP-FC2 -- Natural Polymorphysms -- Synthetic Antagonist with Dominant Negative Effect: PrP-Fc2 -- ANTI-PRP ANTIBODIES WITH ANTI-PRION EFFECTS FOR GENE THERAPY -- CONCLUSION -- REFERENCES -- INDEX.. - GENERATING TRANSMISSIBLE PRION DISEASES DE NOVO -- THE MECHANISM OF TRIGGERING PRION DISEASE BY RPRP AMYLOID FIBRILS -- EXPERIMENTAL EVIDENCE SUPPORT THE SECOND MODEL -- THE HYPOTHESIS OF DEFORMED TEMPLATING -- CONFORMATIONAL SWITCHING WITHIN INDIVIDUAL AMYLOID FIBRILS AND STRAIN ADAPTATION PHENOMENON -- CROSS-TALK BETWEEN AMYLOIDOGENIC PROTEINS -- REFERENCES -- SYNTHETIC PRIONS -- ABSTRACT -- 1. INTRODUCTION -- 2. STRATEGIES FOR DE NOVO MAMMALIAN PRIONS GENERATION -- 2.1. Cell-Free Assay Using Mammalian Prions -- 2.2. De Novo Generation of Prions by Mouse Transgenesis -- 2.3. In Vitro De Novo Prions by PMCA -- 2.4. De Novo Prions by Amyloid Seeding Assay -- 2.5. De Novo Prions by Annealing Technique -- 2.6. Concluding Remarks and New Emerging Techniques for Prion Detection and Generation -- 3. STRUCTURAL BIOLOGY OF NATURAL AND SYNTHETIC PRIONS -- 3.1. Structural Studies on Natural and Synthetic Prions -- 3.2. Lesson from Human Prion Protein Pathological Mutants -- CONCLUSION -- REFERENCES -- RECENT DEVELOPMENTS IN CELL BIOLOGY: ROLE OF GLYCOSYLATION IN PRION DISEASES -- ABSTRACT -- INTRODUCTION -- CONCLUSION -- EXPERIMENTAL PROCEDURES -- Preparation of Brain Homogenate -- Digestion with Proteinase-K (PK) -- Treatment with Guanidine Hydrochloride (GdnHCL) -- Plasma Preparation -- Lectin-ELISA -- Lectin Blotting -- Releasing of PrP from ELISA Plates -- REFERENCES -- TRANSMISSIBILITY OF PRION DISEASES IN RODENT MODELS: ATYPICAL STRAINS -- ABSTRACT -- INTRODUCTION -- The Cattle BSE Strain in the Scrapie Context in Small Ruminants -- Atypical/Nor98 Scrapie in the Scrapie Context -- Atypical BSEs in the Context of BSE -- CONCLUSION -- REFERENCES -- PRE-MORTEM DIAGNOSTIC SCREENING FOR TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES BY PROTEOMIC APPROACHES -- ABSTRACT -- INTRODUCTION -- PROTEOMICS: AN OVERVIEW OF A GROWING FIELD.. - PRION DISEASES: THE AGENT AND THE RESULTING PROTEINOPATHIES -- THE NATURE OF THE INFECTIOUS AGENT -- ANIMAL PRIONOPATHIES -- HUMAN PRIONOPATHIES -- PROTEOMIC AND PRION: A PROMISING PARTNERSHIP -- Pre-Mortem Prion Diagnostic: Screening of Body Fluids and Tissues through Detection of PrP or Surrogate Biomarkers, the Need for Proteomics -- APPLICATION OF PROTEOMICS IN NEURODEGENERATIVE DISEASES -- FUNCTIONS OF THE NATIVE PRPC -- 1. Synaptic Transmission -- 2. Regulation of Copper Metabolism in Vivo and In Vitro -- 3. Cell Adhesion and Signalling -- 4. Apoptotic Pathways -- 5. Redox State of the Cell -- 6. Cell Survival: Protection against Oxidative Stress -- CONCLUSION -- REFERENCES -- ADDED VALUE OF BIOCHEMICAL TOOLS FOR THE DIAGNOSIS OF HUMAN PRION DISEASE -- ABSTRACT -- INTRODUCTION -- DIAGNOSTIC STRATEGY OF PRDS -- 1. Clinical Features -- 2. Electroencephalogram -- 3. Neuroimaging -- 4. Cerebrospinal Fluid: Currently Used in Vivo Biomarkers -- 5. Genetic Analysis -- 6. Tissue-Based Diagnosis -- 7. Diagnosis Criteria of CJD -- THE MOLECULAR DIVERSITY OF PRD, BACKGROUND OF PHENOTYPIC VARIABILITY -- 1. Clinical Variability of Human PrDs -- 2. Molecular Diversity of PrD -- DETECTING DISEASE-ASSOCIATED PRP IN PERIPHERY: THE FUTURE? -- 1. Optimisation of Biochemical Detection of Disease-Associated PrP -- Concentration of PrP -- Partial Purification and/or Concentration of PrPsc -- Alternative Enzymatic Digestion -- Specific Antibodies to PrPsc -- Conformation-Dependent Immunoassay -- Protein Misfolded Cyclic Amplification (PMCA) -- 2. What is Promising for Diagnostic Levels Using PrP Detection? -- 2.1. Peripheral Tissues -- 2.2. Biological fluids : CSF, Blood and Urine -- POTENTIAL DEVELOPMENT OF SURROGATE MARKERS -- 1. New Development in Imaging -- 2. Biological Surrogate Markers: Others Candidates? -- CONCLUSION -- REFERENCES.
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| Emner | |
| Sjanger | |
| ISBN | 9781619427686
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