here - Yale School of Medicine
... out by aging itself (Manuelidis 1994). Such viruses typically originate in the environment. An environmental origin of TSE agents is strongly implicated by the geographic prevalence and origin of particular TSE agents (Manuelidis 2010; Manuelidis et al. 2009a; Manuelidis et al. 2009b) as well as by ...
... out by aging itself (Manuelidis 1994). Such viruses typically originate in the environment. An environmental origin of TSE agents is strongly implicated by the geographic prevalence and origin of particular TSE agents (Manuelidis 2010; Manuelidis et al. 2009a; Manuelidis et al. 2009b) as well as by ...
HOSPITAL EPIDEMIOLOGY AND INFECTION CONTROL POLICIES
... commonly-seen Human Prion Diseases include: sporadic Creutzfeldt-Jakob disease (sHuman Prion Disease), familial Creutzfeldt-Jakob disease (fHuman Prion Disease), and Gertsmann-StrausslerScheinker disease (GSS). IV. PROCEDURES A. Routine Care Of Patients 1. Patients with Human Prion Disease can be ca ...
... commonly-seen Human Prion Diseases include: sporadic Creutzfeldt-Jakob disease (sHuman Prion Disease), familial Creutzfeldt-Jakob disease (fHuman Prion Disease), and Gertsmann-StrausslerScheinker disease (GSS). IV. PROCEDURES A. Routine Care Of Patients 1. Patients with Human Prion Disease can be ca ...
The unique proline-rich domain of parotid proline
... Several efforts have been made to identify structural determinants within secretory proteins that may function in sorting. Among endocrine and exocrine proteins, structural regions have been identified that are either necessary or sufficient for targeting to granules (Stoller and Shields, 1989; Chu ...
... Several efforts have been made to identify structural determinants within secretory proteins that may function in sorting. Among endocrine and exocrine proteins, structural regions have been identified that are either necessary or sufficient for targeting to granules (Stoller and Shields, 1989; Chu ...
Prions as protein-based genetic elements. - Lindquist Lab
... INTRODUCTION Prion proteins are unique because they adopt at least two distinct conformational states, one of which, the prion form, can stimulate the nonprion conformation to convert into the prion form. The term “prion” first described the unusual proteinaceous infectious agent that causes devasta ...
... INTRODUCTION Prion proteins are unique because they adopt at least two distinct conformational states, one of which, the prion form, can stimulate the nonprion conformation to convert into the prion form. The term “prion” first described the unusual proteinaceous infectious agent that causes devasta ...
Creutzfeldt-Jakob disease (CJD) is an infectious, progressive
... a) genetic, b) infectious, and c) sporadic4.1Fivepercent to 15% of cases are thought to be familial, caused by a mutation of the gene encoding the cellular protein on the 20fhchromosome.5This mutation has been postulated to cause the cellular proteins to mimic and convert to the prion protein and ac ...
... a) genetic, b) infectious, and c) sporadic4.1Fivepercent to 15% of cases are thought to be familial, caused by a mutation of the gene encoding the cellular protein on the 20fhchromosome.5This mutation has been postulated to cause the cellular proteins to mimic and convert to the prion protein and ac ...
Document
... • Infectious diseases are spread by respiratory route, direct contact, fecal-oral route, body fluids, and insects • The spread of infectious diseases can be decreased by hygiene, immunization, environmental controls, and healthy lifestyle ...
... • Infectious diseases are spread by respiratory route, direct contact, fecal-oral route, body fluids, and insects • The spread of infectious diseases can be decreased by hygiene, immunization, environmental controls, and healthy lifestyle ...
1. BSE, "Mad Cow" Disease - Cité des Sciences et de l`Industrie
... disease with no need of a virus or any another micro-organism. This approach is very radical, for it goes against everything that has been known till now in biology. This model is globally accepted, but there is no definitive proof to support it. Some research teams continue to think that there is a ...
... disease with no need of a virus or any another micro-organism. This approach is very radical, for it goes against everything that has been known till now in biology. This model is globally accepted, but there is no definitive proof to support it. Some research teams continue to think that there is a ...
genetics of scrapie resistance in sheep
... This is a major area of research, and some promising live-animal tests are in the development and evaluation stages. The scrapie agent is thought to be spread most often from ewe to offspring and to other lambs in contemporary lambing groups through contact of lambs with the placenta and placental ...
... This is a major area of research, and some promising live-animal tests are in the development and evaluation stages. The scrapie agent is thought to be spread most often from ewe to offspring and to other lambs in contemporary lambing groups through contact of lambs with the placenta and placental ...
Research Project Final Report
... successfully applied to the Rov9 cells and subclones more sensitive to natural scrapie infection were isolated; this increased sensitivity may be due to the increased proportion of cells expressing membrane bound PrPC seen in the subclones. However, subcloning of the Rov9 cell line did not increase ...
... successfully applied to the Rov9 cells and subclones more sensitive to natural scrapie infection were isolated; this increased sensitivity may be due to the increased proportion of cells expressing membrane bound PrPC seen in the subclones. However, subcloning of the Rov9 cell line did not increase ...
Prion protein in cardiac muscle of elk (Cervus elaphus nelsoni) and
... 1996; Sigurdson et al., 1999, 2001; Miller & Williams, 2002; Spraker et al., 2002b), but recent investigations have also demonstrated the presence of PrPd in skeletal muscles of TSE-infected laboratory mice and hamsters, of sheep, deer and humans (Bosque et al., 2002; Bartz et al., 2003; Glatzel et ...
... 1996; Sigurdson et al., 1999, 2001; Miller & Williams, 2002; Spraker et al., 2002b), but recent investigations have also demonstrated the presence of PrPd in skeletal muscles of TSE-infected laboratory mice and hamsters, of sheep, deer and humans (Bosque et al., 2002; Bartz et al., 2003; Glatzel et ...
Exotic disease focus: Chronic wasting disease
... (1) McIntyre L. Reports from MAF Biosecurity New Zealand – TSE programme. Surveillance 35(2), ...
... (1) McIntyre L. Reports from MAF Biosecurity New Zealand – TSE programme. Surveillance 35(2), ...
Studies on the transmission of natural scrapie in an infected flock
... Scrapie is a fatal disease that affects the brain of sheep and goats. This disease has been endemic in the National sheep flocks for over 250 years and is become known as classical scrapie. The disease is similar to that of BSE (Bovine Spongiform Encephalopathy – Mad Cows Disease) which affects catt ...
... Scrapie is a fatal disease that affects the brain of sheep and goats. This disease has been endemic in the National sheep flocks for over 250 years and is become known as classical scrapie. The disease is similar to that of BSE (Bovine Spongiform Encephalopathy – Mad Cows Disease) which affects catt ...
Chapt 14 Pathological Protein plus
... Considering that it took the Vatican 359 years to admit that the earth revolves around the sun (1), we should accept with equanimity that Igor V. Zaitsev and Laura Manuelidis are unwilling to relinquish their belief that the TSE agent is a virus……….But, as Max Planck observed (7), “a scientific trut ...
... Considering that it took the Vatican 359 years to admit that the earth revolves around the sun (1), we should accept with equanimity that Igor V. Zaitsev and Laura Manuelidis are unwilling to relinquish their belief that the TSE agent is a virus……….But, as Max Planck observed (7), “a scientific trut ...
CHRONIC WASTING DISEASE (CWD)
... No case of CWD has been reported in any cervid species (neither in enclosures/ zoos nor from free-living cervids) within Europe. However, it is theoretically possible that CWD can also affect European cervids. Research and surveillance programs on CWD did not exist until recently in free-living or c ...
... No case of CWD has been reported in any cervid species (neither in enclosures/ zoos nor from free-living cervids) within Europe. However, it is theoretically possible that CWD can also affect European cervids. Research and surveillance programs on CWD did not exist until recently in free-living or c ...
Identification of a novel Endoplasmic Reticulum Stress Response
... Strausser syndrome (GSS). In cervids, a current area of concern is Chronic Wasting Disease, which affects deer and moose in the western part of North America. While each prion disease presents different symptoms, all are neurodegenerative diseases which result in death (Norrby, 2011). One of the mos ...
... Strausser syndrome (GSS). In cervids, a current area of concern is Chronic Wasting Disease, which affects deer and moose in the western part of North America. While each prion disease presents different symptoms, all are neurodegenerative diseases which result in death (Norrby, 2011). One of the mos ...
Creutzfeldt-Jakob Disease: Recommendations for
... CJD is the most prevalent form of TSEs in humans (table 1). CJD is manifested clinically as a rapidly progressive dementia (cognitive imbalance) that includes psychiatric and behavioral abnormalities, coordination deficits, myoclonus, and a distinct triphasic and polyphasic electroencephalogram read ...
... CJD is the most prevalent form of TSEs in humans (table 1). CJD is manifested clinically as a rapidly progressive dementia (cognitive imbalance) that includes psychiatric and behavioral abnormalities, coordination deficits, myoclonus, and a distinct triphasic and polyphasic electroencephalogram read ...
An Analysis of Risks Associated with the Disposal of Deer
... incorporated in with other waste at the landfill. Landfills generate a certain amount of liquid, termed leachate, which is collected and processed. This leachate results primarily from precipitation falling on the landfill surface. Composite landfill liners prevent leachate from entering groundwater ...
... incorporated in with other waste at the landfill. Landfills generate a certain amount of liquid, termed leachate, which is collected and processed. This leachate results primarily from precipitation falling on the landfill surface. Composite landfill liners prevent leachate from entering groundwater ...
Detecting Mad Cow Disease
... • It is formally known as bovine spongiform encephalopathy (BSE). • BSE causes serious brain damage and death. It is triggered by abnormal proteins called prions, which are not stopped by most disinfection methods. ...
... • It is formally known as bovine spongiform encephalopathy (BSE). • BSE causes serious brain damage and death. It is triggered by abnormal proteins called prions, which are not stopped by most disinfection methods. ...
Combating Infections
... • A prion is an infectious particle (not a cell) made from an abnormally folded protein found on the surfaces of nerve cells. They are not classified into a Domain or Kingdom of living organisms. There is controversy over whether to classify them as microbes, but they are infectious agents. • Prions ...
... • A prion is an infectious particle (not a cell) made from an abnormally folded protein found on the surfaces of nerve cells. They are not classified into a Domain or Kingdom of living organisms. There is controversy over whether to classify them as microbes, but they are infectious agents. • Prions ...
Chronic Wasting Disease Fact Sheet
... agent of TSEs (including CWD) is a misfolded protein called a prion. All mammals produce normal cellular prion protein (abbreviated PrPC). PrPC is a small (approximately 250 amino acid chain), soluble, relatively unstable cell surface protein that is produced by a variety of cell types, but especial ...
... agent of TSEs (including CWD) is a misfolded protein called a prion. All mammals produce normal cellular prion protein (abbreviated PrPC). PrPC is a small (approximately 250 amino acid chain), soluble, relatively unstable cell surface protein that is produced by a variety of cell types, but especial ...
Heat Shock Proteins and Neurodegenerative Disorders
... of affected individuals. It is thought that cellular toxicity is conferred either by PrPSc aggregates or, more likely, by the process of their formation[13]. In scrapie, amyloidogenic proteins such as PrPSc have been postulated to cause neuronal injury and, subsequently, death. Elevated HSP72 levels ...
... of affected individuals. It is thought that cellular toxicity is conferred either by PrPSc aggregates or, more likely, by the process of their formation[13]. In scrapie, amyloidogenic proteins such as PrPSc have been postulated to cause neuronal injury and, subsequently, death. Elevated HSP72 levels ...
Mad Cow Disease - Faculty Website Listing
... • The infectious agent that is widely thought to cause disease is the prion (although that is speculative – there is a lack of evidence), a particle of clumped-up protein. • In its normal form, the prion protein is found in a wide variety of tissues throughout the body, including the brain, immune s ...
... • The infectious agent that is widely thought to cause disease is the prion (although that is speculative – there is a lack of evidence), a particle of clumped-up protein. • In its normal form, the prion protein is found in a wide variety of tissues throughout the body, including the brain, immune s ...
An Orthodox Heresy: Scientific Rhetoric and the Science of Prions.
... Stanley B. Prusiner proposed in April 1982 (in Science) a protein-only hypothesis to explain causation in scrapie, a neurodegenerative disease in sheep. The causative agent, he proposed, is a ubiquitous mammalian protein that either spontaneously or through genetic mutation becomes an errant particl ...
... Stanley B. Prusiner proposed in April 1982 (in Science) a protein-only hypothesis to explain causation in scrapie, a neurodegenerative disease in sheep. The causative agent, he proposed, is a ubiquitous mammalian protein that either spontaneously or through genetic mutation becomes an errant particl ...
Disease name
... Cervid genotyping for CWD resistance/susceptibility: Susceptibility of cervids to CWD infection has been shown to be associated with polymorphisms in the host prion protein gene. Work is in progress to validate recently developed high throughput methods to detect the allelic variants of elk, white-t ...
... Cervid genotyping for CWD resistance/susceptibility: Susceptibility of cervids to CWD infection has been shown to be associated with polymorphisms in the host prion protein gene. Work is in progress to validate recently developed high throughput methods to detect the allelic variants of elk, white-t ...
Veterinary Student Internship Program (VSIP)
... Initial epidemiological studies on the UK BSE epidemic identified the feeding of either tainted milk replacer or other protein supplements to young calves prior to weaning as the principal source of BSE infection (Wilesmith et al., 1992) and all BSE challenge experiments utilize young animals (Well ...
... Initial epidemiological studies on the UK BSE epidemic identified the feeding of either tainted milk replacer or other protein supplements to young calves prior to weaning as the principal source of BSE infection (Wilesmith et al., 1992) and all BSE challenge experiments utilize young animals (Well ...
Prion
A prion (/ˈpriːɒn/) is a protein that can fold in multiple, structurally distinct ways, at least one of which is transmissible to other prion proteins. It is this form of replication that leads to disease that is similar to viral infection. The word prion, coined in 1982 by Stanley B. Prusiner, is short for “proteinaceous infectious particle” derived from the words protein and infection, in reference to a prion's ability to self-propagate and transmit its conformation to other prions. While several yeast proteins have been identified as having prionogenic properties, the first prion protein was discovered in mammals and is referred to as the major prion protein (PrP). This infectious agent causes mammalian transmissible spongiform encephalopathies, including bovine spongiform encephalopathy (BSE, also known as ""mad cow disease"") and scrapie in sheep. In humans, PrP causes Creutzfeldt-Jakob Disease (CJD), variant Creutzfeldt-Jakob Disease (vCJD), Gerstmann–Sträussler–Scheinker syndrome, Fatal Familial Insomnia and kuru.A protein as an infectious agent stands in contrast to all other known infectious agents, like viruses, bacteria, fungi, or parasites—all of which must contain nucleic acids (either DNA, RNA, or both). All known prion diseases in mammals affect the structure of the brain or other neural tissue and all are currently untreatable and universally fatal.Prions are not considered living organisms because they are misfolded protein molecules which may propagate by transmitting a misfolded protein state. If a prion enters a healthy organism, it induces existing, properly folded proteins to convert into the misfolded prion form. In this way, the prion acts as a template to guide the misfolding of more proteins into prion form. In yeast, this refolding is assisted by chaperone proteins such as Hsp104p. These refolded prions can then go on to convert more proteins themselves, leading to a chain reaction resulting in large amounts of the prion form. All known prions induce the formation of an amyloid fold, in which the protein polymerises into an aggregate consisting of tightly packed beta sheets. Amyloid aggregates are fibrils, growing at their ends, and replicate when breakage causes two growing ends to become four growing ends. The incubation period of prion diseases is determined by the exponential growth rate associated with prion replication, which is a balance between the linear growth and the breakage of aggregates. (Note that the propagation of the prion depends on the presence of normally folded protein in which the prion can induce misfolding; animals that do not express the normal form of the prion protein can neither develop nor transmit the disease.)Prion aggregates are extremely stable and accumulate in infected tissue, causing tissue damage and cell death. This structural stability means that prions are resistant to denaturation by chemical and physical agents, making disposal and containment of these particles difficult. Prion structure varies slightly between species, but nonetheless prion replication is subject to occasional epimutation and natural selection just like other forms of replication.