FRI BRIEFINGS Bovine Spongiform Encephalopathy
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A Review on Kuru: Fatal Neurodenegerative Disease”
IOSR Journal Of Pharmacy And Biological Sciences (IOSR-JPBS) e-ISSN:2278-3008, p-ISSN:2319-7676. Volume 15, Issue 3 Ser. II (May –June 2020), PP 10-15 www.Iosrjournals.Org A Review on Kuru: Fatal Neurodegenerative Disease Dr.Velicharla Raviteja1, Ms SaiSreeja Meka2 Department Of Pharm. D, Holy Mary Institute Of Technology And Science(College Of Pharmacy), Keesara, Hyderabad, Telangana-501 301 Abstract: Kuru, a very rare fatal neurodegenerative disease. It mainly affects the cerebellar region of the brain. It is caused by infectious proteins called prions. These prions are a deviant form of harmless protein. Kuru is a transmissible spongiform encephalopathy, prion disease. It is a human prion disease. The symptoms include tremors, loss of coordination, depression, muscle spasms and behavioural changes and ultimately lead to death. The incubation period of this disease varies from person to person and it could be as short as 5years and could be as long as 50 years. It is most prevalently seen in Papua New Guinea where a ritual of cannibalism is practiced, where most of the elderly women and children are affected as they are the one to whom human brain is given to consume during cannibalism. The neuropathological changes are observed on brain tissue during biopsy or after death. There is no treatment for this disease only supportive therapy is given. Moreover, this disease is almost vanished as there are no cases reported in recent years. The practice of cannibalism ritual was also banned by Australian government in 1960s. Keywords:kuru, neurodegenerative disease, prions, transmissible spongiform encephalopathy, tremors, cannibalism, biopsy. -
Transmissibility Versus Pathogenicity of Self-Propagating Protein Aggregates
viruses Review Transmissibility versus Pathogenicity of Self-Propagating Protein Aggregates Byron Caughey 1,* and Allison Kraus 2,* 1 Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Hamilton, MT 59840, USA 2 Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA * Correspondence: [email protected] (B.C.); [email protected] (A.K.); Tel.: +1-406-363-9264 (B.C.) Received: 26 September 2019; Accepted: 6 November 2019; Published: 9 November 2019 Abstract: The prion-like spreading and accumulation of specific protein aggregates appear to be central to the pathogenesis of many human diseases, including Alzheimer’s and Parkinson’s. Accumulating evidence indicates that inoculation of tissue extracts from diseased individuals into suitable experimental animals can in many cases induce the aggregation of the disease-associated protein, as well as related pathological lesions. These findings, together with the history of the prion field, have raised the questions about whether such disease-associated protein aggregates are transmissible between humans by casual or iatrogenic routes, and, if so, do they propagate enough in the new host to cause disease? These practical considerations are important because real, and perhaps even only imagined, risks of human-to-human transmission of diseases such as Alzheimer’s and Parkinson’s may force costly changes in clinical practice that, in turn, are likely to have unintended consequences. The prion field has taught us that a single protein, PrP, can aggregate into forms that can propagate exponentially in vitro, but range from being innocuous to deadly when injected into experimental animals in ways that depend strongly on factors such as conformational subtleties, routes of inoculation, and host responses. -
Genetic Studies in Relation to Kuru: an Overview
Current Molecular Medicine 2004, 4, 375-384 375 Genetic Studies in Relation to Kuru: An Overview L.G. Goldfarb*, L. Cervenakova and D.C. Gajdusek National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA American Red Cross J .H . Holland Laboratory , Rockville, MD 20855, USA Centre National de la Recharche Scientifique, Institut Alfred Fessard, Gif-sur-Yvette, France Abstract: Kuru is a subacute neurodegenerative disease presenting with limb ataxia, dysarthria, and a shivering tremor. The disease progress to complete motor and mental incapacity and death within 6 to 24 months. Neuropathologically, a typical pattern of neuronal loss, astrocytic and microglial proliferation, characteristic “kuru-type” amyloid plaques, and PrP deposits in the cerebral cortex and cerebellum are observed. Kuru is the prototype of a group of human transmissible spongiform encephalopathies (TSEs), or “prion” diseases, that include hereditary, sporadic and infectious forms. The latest member of this group, the variant Creutzfeldt-Jakob disease (vCJD), linked to transmission of bovine spongiform encephalopathy (BSE) to humans, shows features similar to kuru. Kuru has emerged at the beginning of the 1900s in a small indigenous population of New-Guinean Eastern Highlands, reached epidemic proportions in the mid-1950s and disappeared progressively in the latter half of the century to complete absence at the end of the 1990s. Early studies made infection, the first etiologic assumption, seem unlikely and led to a hypothesis that kuru might be a genetically determined or genetically mediated illness. After transmissibility of kuru had been discovered and all major epidemiologic phenomena adequately explained by the spread of an infectious agent with long incubation period through the practice of cannibalism, the pattern of occurrence still continued to suggest a role for genetic predisposition. -
Philly Cheesesteak Sloppy Joe Lesson Plan
GiGi’s Kitchen Purposeful Programs Philly Cheesesteak Sloppy Joe Lesson Plan Philly Cheesesteak Sloppy Joe INGREDIENTS: • 1 pound lean ground beef • 2 tablespoons butter • 1 small yellow onion diced • 1 small green bell pepper diced • 8 ounces brown mushrooms minced • 2 tablespoons ketchup • 1 tablespoon Worcestershire sauce • 1/2 teaspoon Kosher salt • 1/2 teaspoon fresh ground black pepper • 1 tablespoon cornstarch • 1 cup beef broth • 8 ounces Provolone Cheese Slices chopped (use 6oz if you don't want it very cheesy) • 6 brioche hamburger buns DIRECTIONS: Note: click on times in the instructions to start a kitchen timer while cooking. 1. Add the ground beef to a large cast iron skillet (this browns very well) and brown until a deep brown crust appears before breaking the beef apart. 2. Stir the ground beef and brown until a deep crust appears on about 50 or so percent of the beef. 3. Remove the beef (you can leave the fat) and add the butter and the onions and bell peppers and mushrooms. 4. Let brown for 1-2 minutes before stirring, then let brown for another 1-2 minutes before stirring again. 5. Add the beef back into the pan. 6. In a small cup mix the beef broth and cornstarch together 7. Add the ketchup, Worcestershire sauce, salt, black pepper, beef broth/cornstarch mixture into the pan. 8. Cook until the mixture is only slightly liquidy (about 75% of the mixture is above liquid), 3-5 minutes. 9. Turn off the heat, add in the provolone cheese. 10. Served on toasted brioche buns. -
Prions in Variably Protease-Sensitive Prionopathy: an Update
Pathogens 2013, 2, 457-471; doi:10.3390/pathogens2030457 OPEN ACCESS pathogens ISSN 2076-0817 www.mdpi.com/journal/pathogens Review Prions in Variably Protease-Sensitive Prionopathy: An Update Wen-Quan Zou 1,2,3,4,5,6,*, Pierluigi Gambetti 1,3, Xiangzhu Xiao 1, Jue Yuan 1, Jan Langeveld 7 and Laura Pirisinu 8 1 Department of Pathology Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA; E-Mails: [email protected] (P.G.); [email protected] (X.X.); [email protected] (J.Y.) 2 Department of Neurology, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA 3 National Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA 4 National Center for Regenerative Medicine, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA 5 The First Affiliated Hospital, Nanchang University, Nanchang 330006, Jiangxi Province, China 6 State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing 100050, China 7 Central Veterinary Institute of Wageningen UR, Lelystad 8200 AB, the Netherlands; E-Mail: [email protected] (J.L.) 8 Department of Veterinary Public Health and Food Safety, Istituto Superiore di Sanità, Viale Regina Elena 299 00161, Rome, Italy; E-Mail: [email protected] (L.P.) * Author to whom correspondence should be addressed; E-Mail: [email protected]; Tel./Fax: +1-216-368-8993/+1-216-368-2546. Received: 12 June 2013; in revised form: 28 June 2013 / Accepted: 2 July 2013 / Published: 5 July 2013 Abstract: Human prion diseases, including sporadic, familial, and acquired forms such as Creutzfeldt-Jakob disease (CJD), are caused by prions in which an abnormal prion protein (PrPSc) derived from its normal cellular isoform (PrPC) is the only known component. -
Breakthroughs in Antemortem Diagnosis of Neurodegenerative Diseases COMMENTARY Glenn C
COMMENTARY Breakthroughs in antemortem diagnosis of neurodegenerative diseases COMMENTARY Glenn C. Tellinga,1 The World Health Organization forecasts that within 2 Abnormal cytoplasmic accumulation of a normally sol- decades neurodegenerative disorders will eclipse can- uble and unfolded protein called α-synuclein is the cer to become the foremost cause of death in the de- hallmark of diseases referred to as synucleinopathies. veloped world after cardiovascular disease. Accurate Neuronal deposition of α-synuclein aggregates in Lewy detection of pathological processes goes hand in hand bodies occurs in Parkinson’s disease (PD) and dementia with the goals of treatment and prevention and, in light with Lewy bodies (DLB). Yet another protein—the prion of their protracted but worsening clinical progression, protein (PrP)—is central to a group of interrelated dis- the earlier a diagnosis can be made the better. How- orders commonly referred to as prion diseases. ever, the challenge underlying accurate detection of Since the concept underlying the diagnostic ap- neurodegenerative diseases during their clinical phase proach taken by Metrick et al. (1) derives from studies is that specific biomarkers are not present at high of prions, it is worth reviewing what we have learned enough concentrations for routine detection in acces- about PrP and the applicability of these findings to sible specimens. Consequently, it has only been pos- other proteopathic diseases. The prion disorders are sible to definitively diagnose these conditions by transmissible neurodegenerative diseases affecting examination of brain pathology after death. The paper animals and humans. The most common human form by Metrick et al. (1) in PNAS addresses the issue of is Creutzfeldt–Jakob disease (CJD) which occurs most improved antemortem biomarker detection for a frequently as a sporadic, rapidly progressive condition spectrum of neurological disorders, using assays of older individuals. -
The Epidemiology of Kuru: Monitoring the Epidemic from Its Peak to Its End Michael P
CORE Metadata, citation and similar papers at core.ac.uk Provided by PubMed Central Phil. Trans. R. Soc. B (2008) 363, 3707–3713 doi:10.1098/rstb.2008.0071 Published online Review The epidemiology of kuru: monitoring the epidemic from its peak to its end Michael P. Alpers1,2,3,* 1Centre for International Health, ABCRC, Shenton Park Campus, Curtin University, GPO Box U1987, Perth, WA 6845, Australia 2MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK 3Papua New Guinea Institute of Medical Research, PO Box 60, Goroka, EHP 441, Papua New Guinea Kuru is a fatal transmissible spongiform encephalopathy restricted to the Fore people and their neighbours in a remote region of the Eastern Highlands of Papua New Guinea. When first investigated in 1957 it was found to be present in epidemic proportions, with approximately 1000 deaths in the first 5 years, 1957–1961. The changing epidemiological patterns and other significant findings such as the transmissibility of kuru are described in their historical progression. Monitoring the progress of the epidemic has been carried out by epidemiological surveillance in the field for 50 years. From its peak, the number of deaths from kuru declined to 2 in the last 5 years, indicating that the epidemic is approaching its end. The mode of transmission of the prion agent of kuru was the local mortuary practice of transumption. The prohibition of this practice in the 1950s led to the decline in the epidemic, which has been prolonged into the present century by incubation periods that may exceed 50 years. -
Genetic Susceptibility, Evolution and the Kuru Epidemic
View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by PubMed Central Phil. Trans. R. Soc. B (2008) 363, 3741–3746 doi:10.1098/rstb.2008.0087 Published online Genetic susceptibility, evolution and the kuru epidemic Simon Mead1, Jerome Whitfield1,2,3, Mark Poulter1, Paresh Shah1, James Uphill1, Jonathan Beck1, Tracy Campbell1, Huda Al-Dujaily1, Holger Hummerich1, Michael P. Alpers1,2,3 and John Collinge1,* 1MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK 2Papua New Guinea Institute of Medical Research, PO Box 60, Goroka, EHP 441, Papua New Guinea 3Centre for International Health, ABCRC, Shenton Park Campus, Curtin University, GPO Box U1987, Perth, WA 6845, Australia The acquired prion disease kuru was restricted to the Fore and neighbouring linguistic groups of the Papua New Guinea highlands and largely affected children and adult women. Oral history documents the onset of the epidemic in the early twentieth century, followed by a peak in the mid- twentieth century and subsequently a well-documented decline in frequency. In the context of these strong associations (gender, region and time), we have considered the genetic factors associated with susceptibility and resistance to kuru. Heterozygosity at codon 129 of the human prion protein gene (PRNP) is known to confer relative resistance to both sporadic and acquired prion diseases. In kuru, heterozygosity is associated with older patients and longer incubation times. Elderly survivors of the kuru epidemic, who had multiple exposures at mortuary feasts, are predominantly PRNP codon 129 heterozygotes and this group show marked Hardy–Weinberg disequilibrium. -
RIVM Report 257851003 / 2001 Risk Assessment of Shiga-Toxin
RIVM report 257851003 / 2001 Risk assessment of Shiga-toxin producing Escherichia coli O157 in steak tartare in the Netherlands M.J. Nauta, E.G. Evers, K. Takumi and A.H. Havelaar This investigation has been performed by order and for the account of the Directory Board of RIVM, within the framework of project 257851, “Exposure modelling of zoonotic agents in the animal production chain”. RIVM, P.O. Box 1, 3720 BA Bilthoven, telephone: 31 - 30 - 274 91 11; telefax: 31 - 30 - 274 29 71 page 2 of 169 RIVM report 257851 003 RIVM report 257851 003 page 3 of 169 Abstract The methodology of quantitative microbiological risk assessment (QMRA), a tool to evaluate food related health risks, is rapidly developing. As a contribution to this development, a QMRA of Shiga-toxin producing E. coli (STEC) O157 in steak tartare in the Netherlands is conducted, using the Modular Process Risk Model (MPRM) concept. STEC O157 has caused a number of large-scale outbreaks in several industrial countries with severe public health consequences, often associated with the consumption of beef products. An exposure model was constructed, covering the whole food pathway from farm animals to human consumption. This model was linked with a newly developed dose response model of STEC O157 based on Japanese outbreak data. It resulted in estimates of steak tartare contamination (prevalence and concentration) and the incidence of STEC O157 associated illness by steak tartare consumption. As in other QMRAs, these estimates are highly uncertain as a consequence of a lack of adequate data all over the food pathway. -
Balancing Selection at the Prion Protein Gene Consistent with Prehistoric Kurulike Epidemics Simon Mead,1 Michael P
Balancing Selection at the Prion Protein Gene Consistent with Prehistoric Kurulike Epidemics Simon Mead,1 Michael P. H. Stumpf,2 Jerome Whitfield,1,3 Jonathan A. Beck,1 Mark Poulter,1 Tracy Campbell,1 James Uphill,1 David Goldstein,2 Michael Alpers,1,3,4 Elizabeth M. C. Fisher,1 John Collinge1* 1Medical Research Council, Prion Unit, and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK. 2Department of Biology (Galton Laboratory), University College London, Gower Street, London WC1E 6BT, UK. 3Institute of Medical Research, Goroka, EHP, Papua New Guinea. 4Curtin University of Technology, Perth, WA, Australia. *To whom correspondence should be addressed. E-mail: [email protected] Kuru is an acquired prion disease largely restricted to the cannibalism imposed by the Australian authorities in the mid- Fore linguistic group of the Papua New Guinea Highlands 1950s led to a decline in kuru incidence, and although rare which was transmitted during endocannibalistic feasts. cases still occur these are all in older individuals and reflect Heterozygosity for a common polymorphism in the the long incubation periods possible in human prion human prion protein gene (PRNP) confers relative diseasekuru has not been recorded in any individual born after the late 1950s (4). resistance to prion diseases. Elderly survivors of the kuru A coding polymorphism at codon 129 of PRNP is a strong epidemic, who had multiple exposures at mortuary feasts, susceptibility factor for human prion diseases. Methionine are, in marked contrast to younger unexposed Fore, homozygotes comprise 37% of the UK population whereas predominantly PRNP 129 heterozygotes. -
Appetizers Return of the Mac Garden-Fresh Salads Homemade Chili & Soup Sandwiches Combos
APPETIZERS Dipping Sauce Choices: Grit Ranch, Honey Mustard, Wing Sauce, BBQ Sauce, Fry Sauce, OR White Gravy ROCKY MOUNTAIN OYSTERS CRISPY FRIED OKRA Sliced and Breaded... You Guessed it... Bull Testicles fried A Southern Favorite $10 crispy $13 CHILI CHEESE FRIES DILL PICKLE FRIES Crispy French Fries Topped with Pork Green Chili, Shredded Battered Dill Pickle Spears $10 Cheddar & Jack Cheese $10 BEER BATTERED ONION RINGS CRISPY BREADED CHICKEN STRIPS Thick-cut, Beer-battered, Fried. Choice of Sauce $10.5 Choice of Homemade Dipping Sauce $13 CHIPS & DIP Bottomless House-fried Corn Tortilla Chips with Salsa $7 Add Cup of Guacamole +$6 Add Cup of Creamy Queso +$6 Add Cup of Pork Green Chili +$6 Add Cup of Beef Chili +$6 RETURN OF THE MAC All Pasta Dishes served with Cup of Soup or Side Salad SIMPLE GOODNESS GROWN-UP MAC & CHEESE Pasta topped with our Roasted Cheddar Sauce sprinkled Our Simple Goodness topped with Fried Chicken Strips & with Shredded Cheddar Jack Cheese $15. Add Sautéed Crumbled Bacon $18 Broccoli $3 Add Choice of Chili $5 GARDEN-FRESH SALADS Add Avocado to any salad $2 Housemade Dressing Choices: Grit Ranch, Balsamic Vinaigrette, Bleu Cheese, 1000 Island, Honey Mustard or Lemon Basil Vinaigrette. **All dressings are Gluten Free. GRILLED STEAK SALAD CLUB SALAD Mixed Greens topped with Grape Tomatoes, Shredded Mixed Greens topped with Shredded Cheddar & Jack Cheddar & Jack Cheese, Avocado, Carrots & a 6oz Top Cheese, Carrot Sticks, Grape Tomatoes, Boiled Egg, Smokey Sirloin Steak $18 Bacon & All Natural, Nitrate Free Turkey Breast and Choice CRANBERRY SALAD of Dressing $15 Mixed Greens, Sugar Roasted Almonds, Mediterranean CRISPY CHICKEN SALAD Feta Cheese & Craisins $14. -
Raw Ground Beef Legal Regulation
Safely Serving Raw Ground Beef Dishes in Ontario Food Service Premises: A Jurisdictional Review of Regulatory Requirements Michael Jensen, Richard Meldrum, Melissa Moos & Ian Young Ryerson University – School of Occupational and Public Health Background Methods to Reduce Risk ▪ Raw ground beef dishes such as steak tartare, kibbeh nayyeh, and kitfo In order to reduce pathogenic bacteria on the meat surface to an acceptable are not made from hamburger but are typically made from ground level it must be seared or blanched, and then trimmed(4,6). Ultimately, the (1) (6) whole cuts of beef from a single animal . cooking process should achieve a 6-log10 reduction of pathogens . ▪ Although there may be less risk associated with consuming raw ground ▪ Searing involves heating oil or fat in pan and cooking each side of the beef from a single cut of beef in comparison to hamburger, which may meat for approximately 60 seconds to ensure the outer surface reaches a Steak Tartare Lebanese Kibbeh Nayyeh (https://www.npr.org/sections/thesalt/2013/02/06/171301501/w (http://allrecipes.co.uk/recipe/23472/steak-tartare.aspx) (6) be made up of several hundred different animals, there is still a risk of hy-lebanese-love-their-raw-kibbeh) temperature of 75°C for at least 30 seconds . exposure to pathogenic bacteria.(4). ▪ Blanching involves bringing a pot of water to a rolling boil and then ▪ Pathogenic contaminants are generally acquired during the slaughtering submerging the whole cut of beef in the boiling water for approximately process and are found on the outer surface of whole cuts of meat while 60 seconds(6).