4.7 Review

The Zoonotic Potential of Chronic Wasting Disease-A Review

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Review Cell Biology

Non-human primates in prion diseases

Emmanuel E. Comoy et al.

Summary: The use of nonhuman primates as experimental models has played a crucial role in studying prion diseases and providing key insights. These models are still essential for assessing human health risks and also contribute to research on other neurodegenerative diseases associated with prion-like entities.

CELL AND TISSUE RESEARCH (2023)

Article Immunology

Humanized Transgenic Mice Are Resistant to Chronic Wasting Disease Prions From Norwegian Reindeer and Moose

Jonathan D. F. Wadsworth et al.

Summary: This study investigated the zoonotic potential of CWD-infected Norwegian reindeer and moose by inoculating their brain tissue into transgenic mice overexpressing human prion protein. The results showed no evidence of prion transmission, indicating a low zoonotic potential for these isolates.

JOURNAL OF INFECTIOUS DISEASES (2022)

Article Immunology

North American and Norwegian Chronic Wasting Disease Prions Exhibit Different Potential for Interspecies Transmission and Zoonotic Risk

Sandra Pritzkow et al.

Summary: This study investigates the spillover and potential zoonotic transmission of chronic wasting disease (CWD) from various species of cervids. The results suggest that Norwegian CWD prions have a higher potential to infect other animals, while North American CWD prions are more prone to generate human prions.

JOURNAL OF INFECTIOUS DISEASES (2022)

Article Infectious Diseases

Chronic wasting disease in Norway-A survey of prion protein gene variation among cervids

Mariella Evelyn Guere et al.

Summary: The susceptibility of cervids to Chronic Wasting Disease (CWD) in Norway is modulated by variations in the prion protein gene (PRNP), with different species having different levels of susceptibility. The PRNP genetic data indicate considerable susceptibility to CWD among Norwegian cervids, and suggest that PRNP homozygosity may be a risk factor for the atypical CWD observed in moose. The CWD isolates found in Norwegian cervids differ from those previously found in Canada and USA, indicating potential strain dynamics related to PRNP variability.

TRANSBOUNDARY AND EMERGING DISEASES (2022)

Article Biochemistry & Molecular Biology

Strain Typing of Classical Scrapie and Bovine Spongiform Encephalopathy (BSE) by Using Ovine PrP (ARQ/ARQ) Overexpressing Transgenic Mice

Olanrewaju Fatola et al.

Summary: In this study, the incubation period, lesion profile, and PrPSc profile induced by well-defined sheep scrapie isolates, bovine spongiform encephalopathy (BSE), and ovine BSE were investigated in two lines of ovine PrP overexpressing transgenic mice. The results showed genotype-dependent differences between ARQ and VRQ isolates, and BSE had a longer incubation period in Tgshp XI compared to Tgshp IX. Immunohistochemical analysis revealed distinguishing patterns in certain brain regions for both BSE isolates, differentiating them from scrapie.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2022)

Article Neurosciences

Sporadic Creutzfeldt-Jakob disease VM1: phenotypic and molecular characterization of a novel subtype of human prion disease

Ellen Gelpi et al.

Summary: This study identified a new subtype of sCJD with clinicopathological features significantly overlapping with those of the VV1 subtype, possibly representing the missing evidence of V1 sCJD strain propagation in the 129MV host genotype.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2022)

Article Clinical Neurology

Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD

Samia Hannaoui et al.

Summary: This study provides evidence for the zoonotic potential of CWD prions and its distinct clinical presentation and characteristics compared to other prion diseases. The experiments conducted on mice showed that CWD prions can be infectious in the brain and feces, without classical neuropathological changes. These findings pose challenges for current diagnostics and have implications for public health.

ACTA NEUROPATHOLOGICA (2022)

Review Agriculture, Dairy & Animal Science

Classical and Atypical Scrapie in Sheep and Goats. Review on the Etiology, Genetic Factors, Pathogenesis, Diagnosis, and Control Measures of Both Diseases

Cristina Acin et al.

Summary: Prion diseases, such as scrapie, are fatal neurodegenerative diseases caused by a conformational change from PrPC to PrPSc. Understanding the etiology, transmission pathways, tissue infectivity, and detection methods is crucial for disease control.

ANIMALS (2021)

Article Veterinary Sciences

First Detection of Chronic Wasting Disease in Moose (Alces alces) in Sweden

Erik O. Ågren et al.

JOURNAL OF WILDLIFE DISEASES (2021)

Review Clinical Neurology

The importance of ongoing international surveillance for Creutzfeldt-Jakob disease

Neil Watson et al.

Summary: Despite the decreasing incidence of variant Creutzfeldt-Jakob disease, evidence suggests that patients with different forms of human prion diseases still carry a theoretical risk of transmission, highlighting the continued importance of monitoring for Creutzfeldt-Jakob disease.

NATURE REVIEWS NEUROLOGY (2021)

Article Clinical Neurology

Phenotypic diversity of genetic Creutzfeldt-Jakob disease: a histo-molecular-based classification

Simone Baiardi et al.

Summary: The current classification of sporadic Creutzfeldt-Jakob disease (sCJD) includes six major clinicopathological subtypes based on the physicochemical properties of the protease-resistant core of the pathologic prion protein (PrPSc), defining two major PrPSc types (1 and 2), and the codon 129 genotype on the prion protein gene (PRNP). The research analyzed 208 individuals affected by genetic CJD (gCJD), identifying six major groups based on the combination of PrPSc type and codon 129 genotype on PRNP mutated allele. The phenotypic variability of gCJD is mostly consistent with that previously found in sCJD.

ACTA NEUROPATHOLOGICA (2021)

Article Microbiology

Adaptive selection of a prion strain conformer corresponding to established North American CWD during propagation of novel emergent Norwegian strains in mice expressing elk or deer prion protein

Jifeng Bian et al.

Summary: The study shows that CWD in Norwegian reindeer and moose is caused by novel prion strains with distinct properties from North American CWD. These Norwegian prions have the potential to produce adapted strains with improved fitness for contagious transmission. This finding has implications for risk analyses and management of emergent European CWD.

PLOS PATHOGENS (2021)

Article Neurosciences

Prion strains associated with iatrogenic CJD in French and UK human growth hormone recipients

Jean-Yves Douet et al.

Summary: The treatment with human pituitary-derived growth hormone (hGH) was responsible for a significant proportion of iatrogenic Creutzfeldt-Jakob disease (iCJD) cases, with France and the UK experiencing the largest numbers. Differences in PRNP codon 129 polymorphisms and estimated incubation periods did not lead to significant differences in the prion strains identified in hGH-iCJD cases from the two countries.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2021)

Review Veterinary Sciences

Chronic wasting disease in Europe: new strains on the horizon

Michael Andreas Tranulis et al.

Summary: Prion diseases are fatal neurodegenerative disorders caused by misfolded prions, which can be transmitted directly or indirectly. CWD cases have been found in deer and elk, and have emerged in Norway and other Nordic countries, posing a threat to European cervid populations.

ACTA VETERINARIA SCANDINAVICA (2021)

Article Biology

The ecology of chronic wasting disease in wildlife

Luis E. Escobar et al.

BIOLOGICAL REVIEWS (2020)

Article Biochemistry & Molecular Biology

Chronic wasting disease (CWD) prion strains evolve via adaptive diversification of conformers in hosts expressing prion protein polymorphisms

Camilo Duque Velasquez et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2020)

Article Biochemistry & Molecular Biology

Cryo-EM structure of a human prion fibril with a hydrophobic, protease-resistant core

Calina Glynn et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2020)

Article Biochemistry & Molecular Biology

Cryo-EM structure of an amyloid fibril formed by full-length human prion protein

Li-Qiang Wang et al.

NATURE STRUCTURAL & MOLECULAR BIOLOGY (2020)

Article Multidisciplinary Sciences

Studies in bank voles reveal strain differences between chronic wasting disease prions from Norway and North America

Romolo Nonno et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2020)

Article Veterinary Sciences

First Detection of Chronic Wasting Disease in a Wild Red Deer (Cervus elaphus) in Europe

Turid Vikoren et al.

JOURNAL OF WILDLIFE DISEASES (2019)

Article Biochemistry & Molecular Biology

The prion protein in neuroimmune crosstalk

Oyvind Salvesen et al.

NEUROCHEMISTRY INTERNATIONAL (2019)

Article Multidisciplinary Sciences

Use of different RT-QuIC substrates for detecting CWD prions in the brain of Norwegian cervids

Edoardo Bistaffa et al.

SCIENTIFIC REPORTS (2019)

Article Microbiology

Assessment of Chronic Wasting Disease Prion Shedding in Deer Saliva with Occupancy Modeling

Kristen A. Davenport et al.

JOURNAL OF CLINICAL MICROBIOLOGY (2018)

Article Virology

Lack of Transmission of Chronic Wasting Disease to Cynomolgus Macaques

Brent Race et al.

JOURNAL OF VIROLOGY (2018)

Article Immunology

Novel Type of Chronic Wasting Disease Detected in Moose (Alces alces), Norway

Laura Pirisinu et al.

EMERGING INFECTIOUS DISEASES (2018)

Article Immunology

Prion Disease in Dromedary Camels, Algeria

Baaissa Babelhadj et al.

EMERGING INFECTIOUS DISEASES (2018)

Article Immunology

Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions

Marcelo A. Barria et al.

EMERGING INFECTIOUS DISEASES (2018)

Article Virology

Prion Strain Characterization of a Novel Subtype of Creutzfeldt-Jakob Disease

Roberta Galeno et al.

JOURNAL OF VIROLOGY (2017)

Article Neurosciences

The Biological Function of the Prion Protein: A Cell Surface Scaffold of Signaling Modules

Rafael Linden

FRONTIERS IN MOLECULAR NEUROSCIENCE (2017)

Article Multidisciplinary Sciences

Detection of Prions in Blood of Cervids at the Asymptomatic Stage of Chronic Wasting Disease

Carlos Kramm et al.

SCIENTIFIC REPORTS (2017)

Letter Immunology

Chronic Wasting Disease Prion Strain Emergence and Host Range Expansion

Allen Herbst et al.

EMERGING INFECTIOUS DISEASES (2017)

Review Biology

The biological function of the cellular prion protein: an update

Marie-Angela Wulf et al.

BMC BIOLOGY (2017)

Article Environmental Sciences

An assessment of the long-term persistence of prion infectivity in aquatic environments

Alba Marin-Moreno et al.

ENVIRONMENTAL RESEARCH (2016)

Article Biochemistry & Molecular Biology

Cross-species transmission of CWD prions

Timothy D. Kurt et al.

Article Veterinary Sciences

First case of chronic wasting disease in Europe in a Norwegian free-ranging reindeer

Sylvie L. Benestad et al.

VETERINARY RESEARCH (2016)

Correction Medicine, Research & Experimental

Human prion protein sequence elements impede cross-species chronic wasting disease transmission (vol 125, pg 1485, 2015)

Timothy D. Kurt et al.

JOURNAL OF CLINICAL INVESTIGATION (2015)

Article Virology

Deer Prion Proteins Modulate the Emergence and Adaptation of Chronic Wasting Disease Strains

Camilo Duque Velasquez et al.

JOURNAL OF VIROLOGY (2015)

Article Multidisciplinary Sciences

Transmission of scrapie prions to primate after an extended silent incubation period

Emmanuel E. Comoy et al.

SCIENTIFIC REPORTS (2015)

Article Agriculture, Dairy & Animal Science

Chronic Wasting Disease of Cervids: Current Knowledge and Future Perspectives

Nicholas J. Haley et al.

ANNUAL REVIEW OF ANIMAL BIOSCIENCES, VOL 3 (2015)

Article Biotechnology & Applied Microbiology

Quantitative assessment of prion infectivity in tissues and body fluids by real-time quaking-induced conversion

Davin M. Henderson et al.

JOURNAL OF GENERAL VIROLOGY (2015)

Article Veterinary Sciences

Persistence of ovine scrapie infectivity in a farm environment following cleaning and decontamination

Steve A. C. Hawkins et al.

VETERINARY RECORD (2015)

Article Neurosciences

Prion Transmission Prevented by Modifying the β2-α2 Loop Structure of Host PrPC

Timothy D. Kurt et al.

JOURNAL OF NEUROSCIENCE (2014)

Article Immunology

Chronic Wasting Disease Agents in Nonhuman Primates

Brent Race et al.

EMERGING INFECTIOUS DISEASES (2014)

Article Virology

Susceptibility of Domestic Cats to Chronic Wasting Disease

Candace K. Mathiason et al.

JOURNAL OF VIROLOGY (2013)

Article Multidisciplinary Sciences

Rapid Antemortem Detection of CWD Prions in Deer Saliva

Davin M. Henderson et al.

PLOS ONE (2013)

Article Immunology

Foodborne Transmission of Bovine Spongiform Encephalopathy to Nonhuman Primates

Edgar Holznagel et al.

EMERGING INFECTIOUS DISEASES (2013)

Article Biotechnology & Applied Microbiology

Evidence for distinct chronic wasting disease (CWD) strains in experimental CWD in ferrets

Matthew R. Perrott et al.

JOURNAL OF GENERAL VIROLOGY (2012)

Review Clinical Neurology

Review: Creutzfeldt-Jakob disease: prion protein type, disease phenotype and agent strain

M. W. Head et al.

NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY (2012)

Review Biochemistry & Molecular Biology

The role of genetics in chronic wasting disease of North American cervids

Stacie J. Robinson et al.

Article Biochemistry & Molecular Biology

Generation of a New Form of Human PrPSc in Vitro by Interspecies Transmission from Cervid Prions

Marcelo A. Barria et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2011)

Article Biochemistry & Molecular Biology

Real-time quaking-induced conversion A highly sensitive assay for prion detection

Ryuichiro Atarashi et al.

Article Clinical Neurology

Variably Protease-Sensitive Prionopathy: A New Sporadic Disease of the Prion Protein

Wen-Quan Zou et al.

ANNALS OF NEUROLOGY (2010)

Article Biotechnology & Applied Microbiology

Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein

Malin K. Sandberg et al.

JOURNAL OF GENERAL VIROLOGY (2010)

Article Virology

Environmental Sources of Scrapie Prions

Ben C. Maddison et al.

JOURNAL OF VIROLOGY (2010)

Article Biochemical Research Methods

Estimating prion concentration in fluids and tissues by quantitative PMCA

Baian Chen et al.

NATURE METHODS (2010)

Article Biochemistry & Molecular Biology

Prion transmission Prion excretion and occurrence in the environment

Kevin C. Gough et al.

Editorial Material Multidisciplinary Sciences

Prion Strain Mutation and Selection

John Collinge

SCIENCE (2010)

Article Environmental Sciences

Polymorphisms at the PRNP Gene Influence Susceptibility to Chronic Wasting Disease in Two Species of Deer (Odocoileus Spp.) in Western Canada

Gregory A. Wilson et al.

JOURNAL OF TOXICOLOGY AND ENVIRONMENTAL HEALTH-PART A-CURRENT ISSUES (2009)

Article Immunology

Susceptibilities of Nonhuman Primates to Chronic Wasting Disease

Brent Race et al.

EMERGING INFECTIOUS DISEASES (2009)

Article Biochemistry & Molecular Biology

Cell-free propagation of prion strains

Joaquin Castilla et al.

EMBO JOURNAL (2008)

Article Pathology

Experimental chronic wasting disease (CWD) in the ferret

C. J. Sigurdson et al.

JOURNAL OF COMPARATIVE PATHOLOGY (2008)

Article Multidisciplinary Sciences

Global trends in emerging infectious diseases

Kate E. Jones et al.

NATURE (2008)

Letter Biochemical Research Methods

Simplified ultrasensitive prion detection by recombinant PrP conversion with shaking

Ryuichiro Atarashi et al.

NATURE METHODS (2008)

Article Multidisciplinary Sciences

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

Emmanuel E. Comoy et al.

PLOS ONE (2008)

Review Veterinary Sciences

PrP genetics in ruminant transmissible spongiform encephalopathies

Wilfred Goldmann

VETERINARY RESEARCH (2008)

Review Veterinary Sciences

Review on the epidemiology and dynamics of BSE epidemics

Christian Ducrot et al.

VETERINARY RESEARCH (2008)

Article Multidisciplinary Sciences

Prion detection by an amyloid seeding assay

David W. Colby et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2007)

Review Multidisciplinary Sciences

A general model of prion strains and their pathogenicity

John Collinge et al.

SCIENCE (2007)

Article Veterinary Sciences

A natural case of chronic wasting disease in a free-ranging moose (Alces alces shirasi)

Laurie A. Baeten et al.

JOURNAL OF WILDLIFE DISEASES (2007)

Article Biotechnology & Applied Microbiology

Infectious agent of sheep scrapie may persist in the environment for at least 16 years

Gudmundur Georgsson et al.

JOURNAL OF GENERAL VIROLOGY (2006)

Article Veterinary Sciences

Transmission of chronic wasting disease of mule deer to Suffolk sheep following intracerebral inoculation

Amir N. Hamir et al.

JOURNAL OF VETERINARY DIAGNOSTIC INVESTIGATION (2006)

Article Immunology

Human prion disease and relative risk associated with chronic wasting disease

Samantha MaWhinney et al.

EMERGING INFECTIOUS DISEASES (2006)

Article Virology

Transmission of elk and deer prions to transgenic mice

Gultekin Tamguney et al.

JOURNAL OF VIROLOGY (2006)

Article Veterinary Sciences

Oral transmission of chronic wasting disease in captive Shira's moose

Terry J. Kreeger et al.

JOURNAL OF WILDLIFE DISEASES (2006)

Article Pathology

Experimental second passage of chronic wasting disease (CWDmule (deer)) agent to cattle

AN Hamir et al.

JOURNAL OF COMPARATIVE PATHOLOGY (2006)

Article Veterinary Sciences

Additional cases of chronic wasting disease in imported deer in Korea

TY Kim et al.

JOURNAL OF VETERINARY MEDICAL SCIENCE (2005)

Article Medicine, General & Internal

Risk of oral infection with bovine spongiform encephalopathy agent in primates

CI Lasmézas et al.

LANCET (2005)

Review Pathology

Chronic wasting disease

ES Williams

VETERINARY PATHOLOGY (2005)

Article Multidisciplinary Sciences

Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease

C Casalone et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2004)

Article Immunology

Environmental sources of prion transmission in mule deer

MW Miller et al.

EMERGING INFECTIOUS DISEASES (2004)

Review Biochemistry & Molecular Biology

Subclinical prion infection

AF Hill et al.

TRENDS IN MICROBIOLOGY (2003)

Article Veterinary Sciences

Cases of scrapie with unusual features in Norway and designation of a new type, Nor98

SL Benestad et al.

VETERINARY RECORD (2003)

Article Medicine, General & Internal

Other animal prion diseases

CJ Sigurdson et al.

BRITISH MEDICAL BULLETIN (2003)

Article Veterinary Sciences

Chronic wasting disease in deer and elk in North America

ES Williams et al.

REVUE SCIENTIFIQUE ET TECHNIQUE-OFFICE INTERNATIONAL DES EPIZOOTIES (2002)

Article Biotechnology & Applied Microbiology

Distribution and accumulation of PrP in gut-associated and peripheral lymphoid tissue of scrapie-affected Suffolk sheep

R Heggebo et al.

JOURNAL OF GENERAL VIROLOGY (2002)

Article Clinical Neurology

Creutzfeldt-Jakob disease in unusually young patients who consumed venison

ED Belay et al.

ARCHIVES OF NEUROLOGY (2001)

Article Biotechnology & Applied Microbiology

Distribution of the prion protein in sheep terminally affected with BSE following experimental oral transmission

JD Foster et al.

JOURNAL OF GENERAL VIROLOGY (2001)

Article Veterinary Sciences

Epizootiology of chronic wasting disease in free-ranging cervids in Colorado and Wyoming

MW Miller et al.

JOURNAL OF WILDLIFE DISEASES (2000)