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Pathogens
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Chronic Wasting Disease
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Chronic Wasting Disease

A special issue of Pathogens (ISSN 2076-0817).

Deadline for manuscript submissions: 31 May 2024 | Viewed by 9029

Special Issue Editor

Dr. Hyun-Joo Sohn

E-Mail Website
Guest Editor
Foreign Animal Disease Division, Department of Animal and Plant Health Research, Animal and Plant Quarantine Agency, Gimcheon-si 39660, Korea
Interests: chronic wasting disease (CWD)

Special Issue Information

Dear Colleagues,

Chronic Wasting Disease (CWD) is a contagious neurological disease affecting deer, elk and moose and belongs to a group of diseases known as transmissible spongiform encephalopathies (TSEs). It causes a characteristic spongy degeneration of the brains of infected animals resulting in emaciation, abnormal behavior, loss of bodily functions and death.

It is not known exactly how CWD is transmitted. The infectious agent may be passed in feces, urine or saliva. Transmission is thought to be lateral (from animal to animal). Because CWD infectious agents are extremely resistant in the environment, transmission may be both direct and indirect. The apparent persistence of the infectious agents in contaminated environments represents a significant obstacle to eradication of CWD from either captive or free-ranging cervid populations. Cases of CWD occur most commonly in adult animals, but also in yearlings. The disease is progressive and always fatal. Clinical signs of CWD alone are not conclusive. There are other maladies that have symptoms that mimic those of CWD. Currently, the only conclusive diagnosis involves an examination of the brain, tonsils or lymph nodes performed after death.

Despite recent strides in fundamental research and the development of medical countermeasures for some CWD, much remains to be learned about many of these agents to aid in our understanding of in vitro/in vivo prion amplification assays for diagnostic, therapeutic and pathogenic approaches of CWD.

This Special Issue will cover a wide range of topics focusing on CWD and aims to help to enhance current knowledge of TSE and their respective diseases. All types of articles will be considered for publication, including short reports, primary research articles, and reviews.

Dr. Hyun-Joo Sohn
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Pathogens is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2700 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • Prion (BSE, CWD)
  • Chronic wasting disease
  • In vitro amplification
  • Diagnosis
  • Lab animal bioassay
  • Transmission
  • Protein Misfolding cyclic amplification (PMCA)
  • Therapeutics
  • Real time quaking induced conversion (RT-QUIC)
  • Animal models

Published Papers (4 papers)

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Research

16 pages, 1967 KiB  
Article
Standardization of Data Analysis for RT-QuIC-Based Detection of Chronic Wasting Disease
by Gage R. Rowden, Catalina Picasso-Risso, Manci Li, Marc D. Schwabenlander, Tiffany M. Wolf and Peter A. Larsen
Pathogens 2023, 12(2), 309; https://doi.org/10.3390/pathogens12020309 - 13 Feb 2023
Cited by 1 | Viewed by 2034
Abstract
Chronic wasting disease (CWD) is a disease affecting cervids and is caused by prions accumulating as pathogenic fibrils in lymphoid tissue and the central nervous system. Approaches for detecting CWD prions historically relied on antibody-based assays. However, recent advancements in protein amplification technology [...] Read more.
Chronic wasting disease (CWD) is a disease affecting cervids and is caused by prions accumulating as pathogenic fibrils in lymphoid tissue and the central nervous system. Approaches for detecting CWD prions historically relied on antibody-based assays. However, recent advancements in protein amplification technology provided the foundation for a new class of CWD diagnostic tools. In particular, real-time quaking-induced conversion (RT-QuIC) has rapidly become a feasible option for CWD diagnosis. Despite its increased usage for CWD-focused research, there lacks a consensus regarding the interpretation of RT-QuIC data for diagnostic purposes. It is imperative then to identify a standardized and replicable method for determining CWD status from RT-QuIC data. Here, we assessed variables that could impact RT-QuIC results and explored the use of maxpoint ratios (maximumRFU/backgroundRFU) to improve the consistency of RT-QuIC analysis. We examined a variety of statistical analyses to retrospectively analyze CWD status based on RT-QuIC and ELISA results from 668 white-tailed deer lymph nodes. Our results revealed an MPR threshold of 2.0 for determining the rate of amyloid formation, and MPR analysis showed excellent agreement with independent ELISA results. These findings suggest that the use of MPR is a statistically viable option for normalizing between RT-QuIC experiments and defining CWD status. Full article
(This article belongs to the Special Issue Chronic Wasting Disease)
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14 pages, 2802 KiB  
Article
Movement of Chronic Wasting Disease Prions in Prairie, Boreal and Alpine Soils
by Alsu Kuznetsova, Debbie McKenzie, Bjørnar Ytrehus, Kjersti Selstad Utaaker and Judd M. Aiken
Pathogens 2023, 12(2), 269; https://doi.org/10.3390/pathogens12020269 - 07 Feb 2023
Cited by 4 | Viewed by 1822
Abstract
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy negatively impacting cervids on three continents. Soil can serve as a reservoir for horizontal transmission of CWD by interaction with the infectious prion protein (PrPCWD) shed by diseased individuals and from infected [...] Read more.
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy negatively impacting cervids on three continents. Soil can serve as a reservoir for horizontal transmission of CWD by interaction with the infectious prion protein (PrPCWD) shed by diseased individuals and from infected carcasses. We investigated the pathways for PrPCWD migration in soil profiles using lab-scale soil columns, comparing PrPCWD migration through pure soil minerals (quartz, illite and montmorillonite), and diverse soils from boreal (Luvisol, Brunisol) and prairie (Chernozem) regions. We analyzed the leachate of the soil columns by immunoblot and protein misfolding cyclic amplification (PMCA) and detected PrP in the leachates of columns composed of quartz, illite, Luvisol and Brunisol. Animal bioassay confirmed the presence of CWD infectivity in the leachates from quartz, illite and Luvisol columns. Leachates from columns with montmorillonite and prairie Chernozems did not contain PrP detectable by immunoblotting or PMCA; bioassay confirmed that the Chernozemic leachate was not infectious. Analysis of the solid phase of the columns confirmed the migration of PrP to lower layers in the illite column, while the strongest signal in the montmorillonite column remained close to the surface. Montmorillonite, the prevalent clay mineral in prairie soils, has the strongest prion binding ability; by contrast, illite, the main clay mineral in northern boreal and tundra soils, does not bind prions significantly. This suggests that in soils of North American CWD-endemic regions (Chernozems), PrPCWD would remain on the soil surface due to avid binding to montmorillonite. In boreal Luvisols and mountain Brunisols, prions that pass through the leaf litter will continue to move through the soil mineral horizon, becoming less bioavailable. In light-textured soils where quartz is a dominant mineral, the majority of the infectious prions will move through the soil profile. Local soil properties may consequently determine the efficiency of environmental transmission of CWD. Full article
(This article belongs to the Special Issue Chronic Wasting Disease)
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15 pages, 315 KiB  
Article
Assessment of Real-Time Quaking-Induced Conversion (RT-QuIC) Assay, Immunohistochemistry and ELISA for Detection of Chronic Wasting Disease under Field Conditions in White-Tailed Deer: A Bayesian Approach
by Catalina Picasso-Risso, Marc D. Schwabenlander, Gage Rowden, Michelle Carstensen, Jason C. Bartz, Peter A. Larsen and Tiffany M. Wolf
Pathogens 2022, 11(5), 489; https://doi.org/10.3390/pathogens11050489 - 20 Apr 2022
Cited by 4 | Viewed by 2440
Abstract
Chronic wasting disease (CWD) is a transmissible prion disease of the cervidae family. ELISA and IHC tests performed postmortem on the medial retropharyngeal lymph nodes (RPLN) or obex are considered diagnostic gold standards for prion detection. However, differences in CWD transmission, stage of [...] Read more.
Chronic wasting disease (CWD) is a transmissible prion disease of the cervidae family. ELISA and IHC tests performed postmortem on the medial retropharyngeal lymph nodes (RPLN) or obex are considered diagnostic gold standards for prion detection. However, differences in CWD transmission, stage of infection, pathogenesis, and strain can limit performance. To overcome these uncertainties, we used Bayesian statistics to assess the accuracy of RT-QuIC, an increasingly used prion amplification assay, to diagnose CWD on tonsil (TLN), parotid (PLN) and submandibular lymph nodes (SMLN), and ELISA/IHC on RPLN of white-tailed deer (WTD) sampled from Minnesota. Dichotomous RT-QuIC and ELISA/IHC results from wild (n = 61) and captive (n = 46) WTD were analyzed with two-dependent-test, one-population models. RT-QuIC performed on TLN and SMLN of the wild WTD population had similar sensitivity (median range (MR): 92.2–95.1) to ELISA/IHC on RPLN (MR: 91.1–92.3). Slightly lower (4–7%) sensitivity estimates were obtained from farmed animal and PLN models. RT-QuIC specificity estimates were high (MR: 94.5–98.5%) and similar to ELISA/IHC estimates (MR: 95.7–97.6%) in all models. This study offers new insights on RT-QuIC and ELISA/IHC performance at the population level and under field conditions, an important step in CWD diagnosis and management. Full article
(This article belongs to the Special Issue Chronic Wasting Disease)
8 pages, 2444 KiB  
Communication
Chronic Wasting Disease Monitoring in Italy 2017–2019: Neuropathological Findings in Cervids
by Letizia Tripodi, Giuseppe Ru, Fabrizio Lazzara, Lucia Caterina Florio, Cinzia Cocco, Daniela Meloni, Mazza Maria, Elena Bozzetta, Maria Gabriella Perrotta, Maria Caramelli, Cristina Casalone and Barbara Iulini
Pathogens 2022, 11(4), 401; https://doi.org/10.3390/pathogens11040401 - 26 Mar 2022
Cited by 1 | Viewed by 1783
Abstract
Chronic wasting disease (CWD) is a prion disease that affects cervids; it is classified under transmissible spongiform encephalopathies (TSEs). CWD is particularly contagious, making its eradication in endemic areas very difficult and creating serious problems for cervid conservation and breeding. It has recently [...] Read more.
Chronic wasting disease (CWD) is a prion disease that affects cervids; it is classified under transmissible spongiform encephalopathies (TSEs). CWD is particularly contagious, making its eradication in endemic areas very difficult and creating serious problems for cervid conservation and breeding. It has recently become an emerging public health risk to be managed by health authorities. Starting in 2017, active CWD surveillance in Italy has intensified with the monitoring of wild and farmed cervids. The present study summarizes findings from a histopathological survey of the brains from wild ruminants collected via CWD monitoring between 2017 and 2019. A total of 113 brains from 62 red deer (Cervus elaphus) and 51 roe deer (Capreolus capreolus) were submitted for analysis at the National Reference Center for Animal Encephalopathies (CEA) to determine major patterns of neuropathological lesions and correlated pathogens. Brain lesions were detected in 20 animals, 10 brain samples were unsuitable for examination, and 83 presented no lesions. Neuropathological examination revealed non-suppurative encephalitis or meningoencephalitis in most cases (15/20). This brain study revealed evidence for the absence of CWD in Italy and provided a reference spectrum of neuropathological lesions for differential diagnosis in cervids. Full article
(This article belongs to the Special Issue Chronic Wasting Disease)
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