Advancing Prion Science

Advancing Prion Science
Author: Institute of Medicine
Publisher: National Academies Press
Total Pages: 289
Release: 2004-04-04
Genre: Medical
ISBN: 0309090601

In Advancing Prion Science, the Institute of Medicine's Committee on Transmissible Spongiform Encephalopathies Assessment of Relevant Science recommends priorities for research and investment to the Department of Defense's National Prion Research Program (NPRP). Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are invariably fatal neurodegenerative infectious diseases that include bovine spongiform encephalopathy (commonly called mad cow disease), chronic wasting disease, scrapie, and Creutzfeldt-Jakob disease. To develop antemortem diagnostics or therapies for TSEs, the committee concludes that NPRP should invest in basic research specifically to elucidate the structural features of prions, the molecular mechanisms of prion replication, the mechanisms of TSE pathogenesis, and the physiological function of prions' normal cellular isoform. Advancing Prion Science provides the first comprehensive reference on present knowledge about all aspects of TSEsâ€"from basic science to the U.S. research infrastructure, from diagnostics to surveillance, and from prevention to treatment.

Advancing Prion Science

Advancing Prion Science
Author: Institute of Medicine
Publisher: National Academies Press
Total Pages: 125
Release: 2003-04-20
Genre: Medical
ISBN: 0309087449

In Advancing Prion Science, the Institute of Medicine's Committee on Transmissible Spongiform Encephalopathies Assessment of Relevant Science recommends priorities for research and investment to the Department of Defense's National Prion Research Program (NPRP). Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are invariably fatal neurodegenerative infectious diseases that include bovine spongiform encephalopathy (commonly called mad cow disease), chronic wasting disease, scrapie, and Creutzfeldt-Jakob disease. To develop antemortem diagnostics or therapies for TSEs, the committee concludes that NPRP should invest in basic research specifically to elucidate the structural features of prions, the molecular mechanisms of prion replication, the mechanisms of TSE pathogenesis, and the physiological function of prions' normal cellular isoform. Advancing Prion Science provides the first comprehensive reference on present knowledge about all aspects of TSEs' from basic science to the U.S. research infrastructure, from diagnostics to surveillance, and from prevention to treatment. This report summarizes the progress thus far.

Fatal Flaws

Fatal Flaws
Author: Jay Ingram
Publisher: Yale University Press
Total Pages: 294
Release: 2013-03-19
Genre: Medical
ISBN: 0300189893

DIVThe story of the revolutionary science that is unraveling the mysteries of mad cow and other fatal brain diseases/div

Madness and Memory

Madness and Memory
Author: Stanley B. Prusiner
Publisher: Yale University Press
Total Pages: 344
Release: 2014-04-29
Genre: Science
ISBN: 0300191146

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.

Prion Biology

Prion Biology
Author: Stanley B. Prusiner
Publisher:
Total Pages: 0
Release: 2017
Genre: Medical
ISBN: 9781621820932

Prions are infectious proteins responsible for diseases such as Creutzfeldt-Jakob disease and "mad cow" disease. They are misfolded versions of normal proteins that replicate by converting their normal cellular counterparts into abnormal prion proteins that disrupt cell function and can be transmitted to other cells and individuals. This book examines our under-standing of their structure, biochemistry, and pathophysiology.

Prions

Prions
Author: Akikazu Sakudo
Publisher:
Total Pages: 160
Release: 2019-03-12
Genre: Science
ISBN: 9781910190951

Essential reading for everyone working with prions from the PhD student to the experienced scientist.

The Prion Protein

The Prion Protein
Author: Jorg Tatzelt
Publisher:
Total Pages: 80
Release: 2010
Genre: Prions
ISBN: 9780954333522

A conformational transition of the cellular prion protein (PrPC) into an aberrantly folded isoform designated scrapie prion protein (PrPSc) is the hallmark of a variety of neurodegenerative disorders collectively called prion diseases. They include Creutzfeldt-Jakob disease and Gerstmann-Stäussler-Scheinker syndrome in humans, scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and chronic wasting disease (CWD) in free-ranging deer. In contrast to the deadly properties of misfolded PrP, PrPC seems to possess a neuroprotective activity. More-over, animal models indicated that the stress-protective activity of PrPC and the neurotoxic effects of PrPSc are somehow interconnected. In this timely book, leading scientists in the field have come together to highlight the apparently incongruous activities of different PrP conformers. The articles outline current research on celluar pathways implicated in the formation and signaling of neurotoxic and physiological PrP isoforms and delineate future research direction. Topics covered include the physiologcial activity of PrPC and its possible role as a neurotrophic factor, the finding that aberrant PrP conformers can cause neurodegeneration in the absence of infectious prion propagation, the requirement of the GPI anchor of PrPC for the neurotoxic effects of scrapie prions, the pathways implicated in the formation and neurotoxic properties of cytosolically localized PrP, the impact of metal ions on the processing of PrP, and the role of autophagy in the propagation and clearance of PrPSc. The book is fully illustrated and chapters include comprehensive reference sections. Essential reading for scientists involved in prion research.

Prion Biology and Diseases

Prion Biology and Diseases
Author: Stanley B. Prusiner
Publisher: CSHL Press
Total Pages: 1130
Release: 2004
Genre: Medical
ISBN: 9780879696931

This volume is a new edition of the most authoritative book on Prion Biology, first published in 1999 and edited by the Nobel Prize-winning founder of the field. This expanded edition has been completely updated, and includes chapters on therapeutics, and diagnostic methods and approaches.

The Collectors of Lost Souls

The Collectors of Lost Souls
Author: Warwick Anderson
Publisher: JHU Press
Total Pages: 349
Release: 2019-08-27
Genre: Medical
ISBN: 1421433613

This riveting account of medical detective work traces the story of kuru, a fatal brain disease, and the pioneering scientists who spent decades searching for its cause and cure. Winner, William H. Welch Medal, American Association for the History of Medicine Winner, Ludwik Fleck Prize, Society for Social Studies of Science Winner, General History Award, New South Wales Premier's History Awards When whites first encountered the Fore people in the isolated highlands of colonial New Guinea during the 1940s and 1950s, they found a people in the grip of a bizarre epidemic. Women and children succumbed to muscle weakness, uncontrollable tremors, and lack of coordination, until death inevitably supervened. Facing extinction, the Fore attributed their unique and terrifying affliction to a particularly malign form of sorcery. In The Collectors of Lost Souls, Warwick Anderson tells the story of the resilience of the Fore through this devastating plague, their transformation into modern people, and their compelling attraction for a throng of eccentric and adventurous scientists and anthropologists. Battling competing scientists and the colonial authorities, the brilliant and troubled American doctor D. Carleton Gajdusek determined that the cause of the epidemic—kuru—was a new and mysterious agent of infection, which he called a slow virus (now called a prion). Anthropologists and epidemiologists soon realized that the Fore practice of eating their loved ones after death had spread the slow virus. Though the Fore were never convinced, Gajdusek received the Nobel Prize for his discovery. Now revised and updated, the book includes an extensive new afterword that situates its impact within the fields of science and technology studies and the history of science. Additionally, the author now reflects on his long engagement with the scientists and the people afflicted, describing what has happened to them since the end of kuru. This astonishing story links first-contact encounters in New Guinea with laboratory experiments in Bethesda, Maryland; sorcery with science; cannibalism with compassion; and slow viruses with infectious proteins, reshaping our understanding of what it means to do science.

PrPSc Prions: State of the Art

PrPSc Prions: State of the Art
Author: Joaquín Castilla
Publisher: MDPI
Total Pages: 211
Release: 2018-11-07
Genre: Science
ISBN: 3038973084

This book is a printed edition of the Special Issue "PrPSc prions: state of the art" that was published in Pathogens