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The author, a 1997 recipient of the Noble Prize in medicine, describes the years he spent researching and demonstrating how the infectious proteins known as prions were responsible for brain diseases and how his theory has now become widely accepted in the science establishment.
"How the Cows Turned Mad tells the story of a disease that continues to elude on many levels. Yet science has come far in understanding its origins, incubation, and transmission. This book is a case history that illuminates the remarkable progression of science."--BOOK JACKET.
Most textbooks on neurodegenerative disorders have used a classification scheme based upon either clinical syndromes or anatomical distribution of the pathology. In contrast, this book looks to the future and uses a classification based upon molecular mechanisms, rather than clinical or anatomical boundaries. Major advances in molecular genetics and the application of biochemical and immunocytochemical techniques to neurodegenerative disorders have generated this new approach. Throughout most of the current volume, diseases are clustered according to the proteins that accumulate within cells (e.g. tau, α-synuclein and TDP-43) and in the extracellular compartments (e.g. β-amyloid and prion proteins) or according to a shared pathogenetic mechanism, such as trinucleotide repeats, that are a feature of specific genetic disorders. Chapters throughout the book conform to a standard lay-out for ease of access by the reader and are written by a panel of International Experts Since the first edition of this book, major advances have been made in the discovery of common molecular mechanisms between many neurodegenerative diseases most notably in the frontotemporal lobar degenerations (FTLD) and motor neuron disease or amyotrophic lateral sclerosis. This book will be essential reading for clinicians, neuropathologists and basic neuroscientists who require the firm up-to-date knowledge of mechanisms, diagnostic pathology and genetics of Neurodegenerative diseases that is required for progress in therapy and management.
These guidelines provide evidence-based information to national regulatory authorities, especially to those where Bovine Spongiform Encephalopathy (BSE) has not yet been reported and where surveillance systems for BSE and variant Creutzfeldt-Jakob disease (vCJD), the human disease caused by infection with the BSE agent, may not be in place. Preventive measures to minimise the risk of transmitting TSEs to humans from pharmaceutical and biological products in which ruminant animal-derived materials are used and from humans to humans by blood and blood products and by human cells, tissues and organs are discussed.