Download or read book What is Kuru A Deadly First Human Prion misfolded proteins Disease written by Dr. Hakim. K. Saboowala and published by Dr.Hakim Saboowala. This book was released on 2021-04-28 with total page 54 pages. Available in PDF, EPUB and Kindle. Book excerpt: What is Kuru? A Deadly First Human Prion (misfolded proteins)Disease. Kuru is an incurable and fatal neurodegenerative disorder that was formerly common among the Fore people of Papua New Guinea. Kuru, the first human prion disease was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008). The discovery of kuru opened new vistas of human medicine and was pivotal in the subsequent transmission of Creutzfeldt–Jakob disease, as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the 20th century. An attempt is made here to summarize the history of this seminal discovery, epidemiology, clinical picture, neuropathology, investigations, management, complications, prognosis and the descriptions of electron microscopy and confocal microscopy of kuru amyloid plaques along with several illustrations for better understanding of a quaint but interesting entity. …Dr. H. K. Saboowala. M.B.(Bom) .M.R.S.H.(London).
Download or read book Fatal Flaws written by Jay Ingram and published by Yale University Press. This book was released on 2013-03-19 with total page 294 pages. Available in PDF, EPUB and Kindle. Book excerpt: DIVThe story of the revolutionary science that is unraveling the mysteries of mad cow and other fatal brain diseases/div
Download or read book The Prion Protein written by Jorg Tatzelt and published by . This book was released on 2010 with total page 80 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.
Download or read book Neuropathology of Neurodegenerative Diseases written by Gabor G. Kovacs and published by Cambridge University Press. This book was released on 2017-12-13 with total page 320 pages. Available in PDF, EPUB and Kindle. Book excerpt: This practical guide to the diagnosis of neurodegenerative diseases discusses modern molecular techniques, morphological classification, fundamentals of clinical symptomology, diagnostic pitfalls and immunostaining protocols. It is based on the proteinopathy concept of neurodegenerative disease, which has influenced classification and provides new strategies for therapy. Numerous high-quality images, including histopathology photomicrographs and neuroradiology scans, accompany the description of morphologic alterations and interpretation of immunoreactivities. Diagnostic methods and criteria are placed within recent developments in neuropathology, including the now widespread application of immunohistochemistry. To aid daily practice, the guide includes diagnostic algorithms and offers personal insights from experienced experts in the field. Special focus is given to the way brain tissue should be handled during diagnosis. This is a must-have reference for medical specialists and specialist medical trainees in the fields of pathology, neuropathology and neurology working with neuropathologic features of neurodegenerative diseases.
Download or read book Advancing Prion Science written by Institute of Medicine and published by National Academies Press. This book was released on 2003-03-20 with total page 125 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.
Download or read book The Family That Couldn t Sleep written by D. T. Max and published by Random House. This book was released on 2006-09-05 with total page 333 pages. Available in PDF, EPUB and Kindle. Book excerpt: For two hundred years a noble Venetian family has suffered from an inherited disease that strikes their members in middle age, stealing their sleep, eating holes in their brains, and ending their lives in a matter of months. In Papua New Guinea, a primitive tribe is nearly obliterated by a sickness whose chief symptom is uncontrollable laughter. Across Europe, millions of sheep rub their fleeces raw before collapsing. In England, cows attack their owners in the milking parlors, while in the American West, thousands of deer starve to death in fields full of grass. What these strange conditions–including fatal familial insomnia, kuru, scrapie, and mad cow disease–share is their cause: prions. Prions are ordinary proteins that sometimes go wrong, resulting in neurological illnesses that are always fatal. Even more mysterious and frightening, prions are almost impossible to destroy because they are not alive and have no DNA–and the diseases they bring are now spreading around the world. In The Family That Couldn’t Sleep, essayist and journalist D. T. Max tells the spellbinding story of the prion’s hidden past and deadly future. Through exclusive interviews and original archival research, Max explains this story’s connection to human greed and ambition–from the Prussian chemist Justus von Liebig, who made cattle meatier by feeding them the flesh of other cows, to New Guinean natives whose custom of eating the brains of the dead nearly wiped them out. The biologists who have investigated these afflictions are just as extraordinary–for example, Daniel Carleton Gajdusek, a self-described “pedagogic pedophiliac pediatrician” who cracked kuru and won the Nobel Prize, and another Nobel winner, Stanley Prusiner, a driven, feared self-promoter who identified the key protein that revolutionized prion study. With remarkable precision, grace, and sympathy, Max–who himself suffers from an inherited neurological illness–explores maladies that have tormented humanity for centuries and gives reason to hope that someday cures will be found. And he eloquently demonstrates that in our relationship to nature and these ailments, we have been our own worst enemy.
Download or read book Human Prion Diseases written by and published by Elsevier. This book was released on 2018-06-07 with total page 520 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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
Download or read book Neurogenetics Part II written by and published by Elsevier. This book was released on 2018-01-29 with total page 480 pages. Available in PDF, EPUB and Kindle. Book excerpt: Neurogenetics, Part II, Volume 148, the latest release in the Handbook of Clinical Neurology, provides the latest information on the genetic methodologies that are having a significant impact on the study of neurological and psychiatric disorders. Using genetic science, researchers have identified over 200 genes that cause or contribute to neurological disorders. Still an evolving field of study, defining the relationship between genes and neurological and psychiatric disorders is expected to dramatically grow in scope. Part II builds on the foundation of Part I, expanding the coverage to dementias, paroxysmal disorders, neuromuscular disorders, white matter and demyelination diseases, cerebrovascular diseases, adult psychiatric disorders and cancer and phacomatoses. - Contains comprehensive coverage of neurogenetics - Details the latest science and its impact on our understanding of neurological, psychiatric disorders - Presents a focused reference for clinical practitioners and the neuroscience/neurogenetics research community
Download or read book Kuru Sorcery written by Shirley Lindenbaum and published by Routledge. This book was released on 2015-12-03 with total page 241 pages. Available in PDF, EPUB and Kindle. Book excerpt: Perhaps the best-documented epidemic in the history of medicine, kuru has been studied for more than fifty years by international investigators from medicine and the human sciences. This significantly revised edition of the landmark anthropological classic Kuru Sorcery brings up to date the anthropological contribution to understanding disease, the medical research that resulted in two medical Nobel Prizes, and the views of the Fore people who endured the epidemic and who still believe that sorcerers, rather than cannibalism, caused kuru. The kuru epidemic serves as a prism through which to see how Fore notions of disease causation bring into single focus their views about the body, the world of social and spiritual relations, and changes in economic and political conditions-aspects of thought and behaviour that Western medicine keeps separate.
Download or read book PrPSc Prions State of the Art written by Joaquín Castilla and published by MDPI. This book was released on 2018-11-07 with total page 211 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book is a printed edition of the Special Issue "PrPSc prions: state of the art" that was published in Pathogens
Download or read book Prion Diseases of Humans and Animals written by Stanley B. Prusiner and published by Prentice Hall. This book was released on 1992 with total page 628 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Download or read book How the Cows Turned Mad written by Maxime Schwartz and published by Univ of California Press. This book was released on 2004-09-13 with total page 264 pages. Available in PDF, EPUB and Kindle. Book excerpt: "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.
Download or read book Neurodegenerative Diseases written by G. Jolles and published by Academic Press. This book was released on 1994-11-14 with total page 330 pages. Available in PDF, EPUB and Kindle. Book excerpt: Over the last few years, the considerable progress made in biochemistry, virology, molecular biology and genetics has revealed some of the intimate mechanisms of the neurodegenerative diseases. The present volume is an attempt to review the latest data in the field to illuminate new avenues for future research. This volume gathers together chapters and discussions on the etiology and pathogenesis of the neurodegenerative diseases. Apoptosis of programmed cell death as well as other genetic implications are discussed; special attention is given to the coexistence and interconnection of genetic and environmental factors. There is extensive coverage of prions responsible for bovine spongiform encephalopathy, Cruetzfeld-Jacob disease and kuru. The various aspects of non-conventional transmissible agents are thoroughly reviewed. Further contributions deal with the role of growth factors as well as of free radicals. Consideration is given to the molecular mechanisms of Alzheimer's disease, in particular the role of tau protein. Finally, several pharmacological models now available, which throw light upon aspects of Parkinson's disease, Huntington's chorea and multiple sclerosis, are examined and discussed. It is hoped that recent scientific advances will lead to the discovery of new drugs to fill the current therapeutic void. There are hopes of an early indication of this in the case of amyotrophic lateral sclerosis.
Download or read book Slow Latent and Temperate Virus Infections written by U.S. Department of Health, Education, and Welfare and published by . This book was released on 1965 with total page 598 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Download or read book Prion written by Yusuf Tutar and published by BoD – Books on Demand. This book was released on 2017-03-08 with total page 240 pages. Available in PDF, EPUB and Kindle. Book excerpt: Protein aggregation causes malfunction in several biochemical processes. Genetic and spontaneous formations of these transmissible spongiform encephalopathies are fatal to humans and animals. Conformational change of normal form of the protein to misfolded form causes its accumulation. The misfolded infectious protein agent forms the pathogenesis of the disease. This book presents pathology of the disease along with current knowledge of the structure-activity mechanism in the first two sections. Dyshomeostasis of metals is implicated in the pathogenesis of prions, and this influence is discussed further to understand the prion mechanism. Genetic resistance and immunobiology of the disease are elaborated in the following section. Finally, a computational study on the dynamics of the prion propagation provides a structural basis of the mechanism.
Download or read book Neurological Complications of Infectious Diseases written by Rodrigo Hasbun, MD MPH and published by Springer Nature. This book was released on 2020-12-13 with total page 389 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book offers a comprehensive review of the most common infectious diseases that affect the nervous system. Written by international experts, it provides a guide to clinicians for accurately diagnosing and treating these challenging syndromes. Organized into six sections, the book presents didactic, up-to-date information on the following topics relating to central nervous system (CNS) infections: diagnosis and evaluation of the patient, bacterial, viral, fungal and mycobacterial infections, disorders of the spinal cord, and a myriad of miscellaneous infections. Chapters specifically reflect and look to resolve the common obstacles clinicians face in the field, such as having unknown etiologies on the majority of CNS infections, insensitive and slow microbiological techniques, an increasing number of immunosuppressed individuals with atypical presentations and pathogens, and a lack of standardized diagnostic algorithms. A complex yet accessible addition to the Current Clinical Neurology Series, Neurological Complications of Infectious Diseases invaluably examines a wide range of infections that have neurological complications and sequelae.
