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Human Prion Diseases

Human Prion Diseases PDF Author:
Publisher: Elsevier
ISBN: 0444639535
Category : Medical
Languages : en
Pages : 512

Book Description
Human Prion Diseases, Volume 153 is designed to update the reader on the latest advances and clinical aspects of prion diseases. The book is organized into five sections, including the pathophysiology of prions and a description of animal and human diseases. This is followed by detailed reports on recent advances in diagnosis strategies for the development of novel anti-prion molecules and possible designs of clinical trials in such a rare disease. An introductory chapter gives an extensive historical background of prion research, with a final chapter highlighting recent progress, and more importantly, unsolved problems. Offers an authoritative overview of prion diseases in humans, detailing the pathogenesis of the disease, clinical investigations, and the diagnosis of both the genetic and acquired forms Provides clarity and context by presenting prion diseases in relation to other neurodegenerative diseases in humans Emphasizes the unique properties of prion diseases and consequent problems they can cause, both clinically and in public health terms

Human Prion Diseases

Human Prion Diseases PDF Author:
Publisher: Elsevier
ISBN: 0444639535
Category : Medical
Languages : en
Pages : 512

Book Description
Human Prion Diseases, Volume 153 is designed to update the reader on the latest advances and clinical aspects of prion diseases. The book is organized into five sections, including the pathophysiology of prions and a description of animal and human diseases. This is followed by detailed reports on recent advances in diagnosis strategies for the development of novel anti-prion molecules and possible designs of clinical trials in such a rare disease. An introductory chapter gives an extensive historical background of prion research, with a final chapter highlighting recent progress, and more importantly, unsolved problems. Offers an authoritative overview of prion diseases in humans, detailing the pathogenesis of the disease, clinical investigations, and the diagnosis of both the genetic and acquired forms Provides clarity and context by presenting prion diseases in relation to other neurodegenerative diseases in humans Emphasizes the unique properties of prion diseases and consequent problems they can cause, both clinically and in public health terms

Prion Diseases

Prion Diseases PDF Author: John Collinge (MD.)
Publisher:
ISBN:
Category : Medical
Languages : en
Pages : 226

Book Description
The number of neurological conditions associated with the mutant "prion" protein continues to grow. The list includes BSE and scrapie, which affect cattle and sheep respectively, and Creutzfeldt-Jacob Disease, which affects humans. This is an area of intense interest to neuroscientists,veterinary scientists, and clinicians. It has also attracted significant media attention because of the potential risks to humans. This book brings together leading researchers in the field to provide the most up-to-date and authoritative summary available of the field. Contents include human and animal prion diseases; pathology and cell biology of prion diseases; and prion protein structure.

Madness and Memory

Madness and Memory PDF Author: Stanley B. Prusiner
Publisher: Yale University Press
ISBN: 0300191146
Category : Science
Languages : en
Pages : 344

Book Description
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.

Prions

Prions PDF Author: Claudio Soto
Publisher: CRC Press
ISBN: 142004012X
Category : Medical
Languages : en
Pages : 184

Book Description
Prion-related diseases, known as transmissible spongiform encephalopathies (TSEs), are infectious, fatal neurodegenerative disorders for which there is no cure, treatment, nor even a means for early diagnosis. The horrific advent of Mad Cow Disease -- transmitted to humans through eating meat from steers sickened by bovine spongiform encephalopathy

Fatal Protein

Fatal Protein PDF Author: Rosalind M. Ridley
Publisher:
ISBN:
Category : Medical
Languages : en
Pages : 272

Book Description
This book presents a clear and authoritative account of the prion diseases for the non-specialist. It deals with the history of scrapie in sheep, the spread of kuru through cannibalism in Papua New Guinea, BSE, which has killed 170,000 cattle in the UK, and the question of whether the newvariant form of Creutzfeld-Jakob disease (CJD) results from eating BSE-infected meat. All aspects of the diseases are covered, including genetics, clinical aspects, laboratory research, epidemiology, and field studies. Accessibly written by authors who are leading researchers in the field, it is thefirst book to provide a scientifically accurate yet readable account of these topical diseases.

Advancing Prion Science

Advancing Prion Science PDF Author: Institute of Medicine
Publisher: National Academies Press
ISBN: 0309168597
Category : Medical
Languages : en
Pages : 125

Book Description
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.

Neurodegeneration and Prion Disease

Neurodegeneration and Prion Disease PDF Author: David R. Brown
Publisher: Springer Science & Business Media
ISBN: 9780387239224
Category : Medical
Languages : en
Pages : 498

Book Description
This is the first and only book on the subject of prions to cover the cause of cell death in the disease. It covers the full range of competing theories on the subject, from broad description and basic points up to the final details of the basic science.

Prions and Mad Cow Disease

Prions and Mad Cow Disease PDF Author: Brian K. Nunnally
Publisher: CRC Press
ISBN: 0824756843
Category : Medical
Languages : en
Pages : 414

Book Description
The alarm sounded by Canada's recently confirmed case of bovine spongiform encephalopathy (BSE) has reaffirmed the exigency of establishing improved safeguards and more aggressive surveillance protocols in North America and around the world. Research converging on the probable causative agent-prion proteins-calls for intensive assessment of the headway gained in tracing prions, testing for transmissible neurodegenerative diseases, and developing methods for cornering the epidemic. Administered by an illustrious panel of 36 international contributors, this timely book marshals techniques for prion protein assay and diagnosis of transmissible spongiform encephalopathies (TSEs).

The Prion Protein

The Prion Protein PDF Author: Jorg Tatzelt
Publisher:
ISBN: 9780954333522
Category : Prions
Languages : en
Pages : 80

Book Description
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

Prion Biology PDF Author: Stanley B. Prusiner
Publisher: Perspectives Cshl
ISBN: 9781621820932
Category : Medical
Languages : en
Pages : 0

Book Description
"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 exam