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This fully revised edition explores the management of neurological disorders with a focus on neuroprotection, disease modification, and neuroregeneration rather than symptomatic treatment. Since the publication of the first edition, advances in biotechnology, particularly in cell and gene therapies, are reflected in this volume, as are numerous new and repurposed drugs in clinical trials. Overall, The Handbook of Neuroprotection serves as a comprehensive review of neuroprotection based on knowledge of the molecular basis of disorders of the central nervous system. In-depth and authoritative, The Handbook of Neuroprotection, Second Edition features a compendium of vital knowledge aimed at providing researchers with an essential reference for this key neurological area of study.

Provides a timely overview of critical advances in molecular and cellular neurobiology, covers key methodologies driving progress, and highlights key future directions for research on neuronal injury and neurodegeneration relevant to neuronal brain pathologies. The editors bring together contributions from internationally recognized workers in the field to provide an up to date account of how and why molecular and cellular neurobiology is such an important area for clinical neuroscience. Understanding the molecular aspects of a number of neurodegenerative conditions such as Parkinson's or Alzheimer's disease for the purpose of improving patient management remains a major challenge of neurobiology be it from the basic or clinical perspective. A strategic evaluation of research contributions and the power of modern methods will help advance knowledge over the next years.

This unique text introduces students and researchers to the world of misfolded proteins, toxic oligomers, and amyloid assemblages, and the diseases of the brain that result. During the past few years the connections between failures in protein quality control and neurological disorders have been reinforced and strengthened by discoveries on multiple fronts. These findings provide novel insights on how amyloidogenic oligomers and fibrils form, interconvert from one state to another, and propagate from cell to cell and region to region. Starting with protein folding and protein quality control basics, the reader will learn how misfolded proteins can cause diseases ranging from prion diseases to Alzheimer's disease and Parkinson's disease to Huntington's disease, amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Authoritative but written in a clear and engaging style, Fundamentals of Neurodegeneration and Protein Misfolding Disorders addresses one of today's forefront areas of science and medicine. The text emphasizes the new groundbreaking biophysical and biochemical methods that enable molecular-level explorations and the conceptual breakthroughs that result. It contains separate chapters on each of the major disease classes. Special emphasis is placed on those factors and themes that are common to the diseases, especially failures in synaptic transmission, mitochondrial control, and axonal transport; breakdowns in RNA processing; the potential role of environmental factors; and the confounding effects of neuroinflammation. The book is ideal for use in teaching at the advanced undergraduate and graduate levels, and serves as a comprehensive reference for a broad audience of students and researchers in neuroscience, molecular biology, biological physics and biomedical engineering.

Most of the world's experts on prions met for a workshop in Erice in August 1996. The aim of the workshop was to discuss the fundamentals of the science ofprions. It was for­ tunate that so many could be present given the pressure that they were under because of the data presented in March 1996, indicating that Bovine Spongiform Encephalopathy, BSE or Mad Cow Disease, had penetrated the species barrier and was beginning to cause a new dis­ ease in humans--the new variant ofCreutzfeldt-Jakob Disease, nvCJD. This important and urgent subject became an additional major topic at the workshop. This is a book containing most of the talks plus the abstracts of those unable to find time to write up their talks. Almost all papers were written in the spring and summer of 1997 and contain material added after the workshop; thus Bob Will's paper on the new variant of cm contains data up to July 1997 and four contributions arrived in October 1997. In addition to the talks given at our workshop, there was a special joint session with the Planetary Emergencies Workshop where many distinguished scientists, including three Nobel laureates, discussed major issues affecting our planet. Six talks were given by us to this other workshop, five about prions, BSE, and nvCJD, and one on the broader issue of new epi­ demics by Luc Montagnier. Although most of the talks concerned research issues, there were a few special talks.

Prion diseases, also known as Transmissible Spongiform Encephalopathies (TSEs), exist in both humans (Creutzfeldt-Jakob disease (CJD)) and animals (scrapie, bovine spongiform encephalopathy (BSE), chronic wasting disease) and have the unique property of being infectious, sporadic or genetic in origin. Although the precise nature of the infectious agent responsible for TSEs is not definitely identified, it is now clearly demonstrated that a protein named PrP (for Prion Protein) plays a critical role in the transmission and pathogenesis of TSEs. This book provides the general description as well as the details of the techniques currently used for the study of prion diseases. Taking into account the pivotal role played by PrP it is not surprising that many Chapters of this book deal with the purification, the detection and the characterization of the different forms of this protein. In addition, in vitro, cellular and animal models specifically adapted to the study of TSEs, as well as bio-safety procedures are described. Each Chapter is written by scientists involved for many years in their respective domain of prion biology who give the best of their knowledge in this technical document. This volume is a very useful tool for any laboratory which recently decided to contribute to the study of TSEs as well as for teams already engaged in this field for many years but interested in extending their technical capacity toward new methods. Features: Purification of PrPC and the Pathological Isoform of Prion Protein (PrPsc or PrP-res) Animal Models of TSEs Cell Culture Models of TSEs PrPsc Immunohistochemistry Western Immunoblotting Techniques Antibody Production and ELISA TSE Strain Typing in Mice Biosafety and Decontamination Procedures Cell-free Conversion of Prion Proteins Cytotoxicity of PrP Peptides Cyclic Amplification of Prion Protein Misfolding< Of interest to: Researchers and clinicians in the fields of cell biology, biomedicine, neuroscience/neuropathology, veterinary medicine and biochemistry.

It has been recognized that viruses can induce neuronal damage by a variety of mechanisms. This volume summarizes, for the first time, the various ways that neurons can degenerate under the influence of viral infection, ranging from acute necrosis and virus-induced apoptosis to chronic damage in persistent infections. The mechanisms of neuronal latency are dealt with as well. The volume also includes chapters that review the chronic damage caused by viral proteins that interfere with differentiated functions of the neuron, including signaling by neurotransmitters and signal transduction by trophic factors. Other chapters deal with the controversial role of cellular immune reactions, which may be helpful in controlling infection, but may also be detrimental to the host nervous system. The specific mechanisms of damage involved in retroviral infections and in prion diseases of the nervous system are reviewed, and finally how autoimmune diseases can lead to neuronal damage.

Neurobiology of Disease is aimed at any basic scientist or clinician scientist teaching a course or conducting research on the basic science underlying the major neurological diseases. It provides an excellent overview of cutting-edge research on the fundamental disorders of the nervous system, including physiological and molecular aspects of dysfunction. The major categories of neurological disease are covered, and the chapters provide specific information about particular diseases exemplifying each of these categories. Sufficient clinical information is included to put into perspective the basic mechanisms discussed. The book assembles a world-class team of section editors and chapters written by acknowledged experts in their respective fields. - Provides cutting edge information about fundamental mechanisms underlying neurological diseases - Amply supplied with tables, illustrations and references - Includes supporting clinical information putting the mechanisms of disease into perspective