Article
Veterinary Sciences
Tyler A. Harm, Jodi D. Smith, Eric D. Cassmann, Justin J. Greenlee
Summary: The study evaluated the efficacy of different chemical treatments for scrapie inactivation, finding partial reduction in PrPSc immunoreactivity but also an impact on survival rate and incubation periods in experimental sheep.
RESEARCH IN VETERINARY SCIENCE
(2022)
Article
Immunology
Justin J. Greenlee, S. Jo Moore, Eric D. Cassmann, Zoe J. Lambert, Robyn D. Kokemuller, Jodi D. Smith, Robert A. Kunkle, Qingzhong Kong, M. Heather West Greenlee
Summary: The study found that white-tailed deer are susceptible to the classic scrapie agent from sheep, and the molecular profiles of PrPSc in infected deer are difficult to differentiate from those with chronic wasting disease (CWD).
JOURNAL OF INFECTIOUS DISEASES
(2023)
Article
Veterinary Sciences
Timm Konold, John Spiropoulos, Janet Hills, Hasina Abdul, Saira Cawthraw, Laura Phelan, Amy Mckenna, Lauren Read, Sara Canoyra, Alba Marin-Moreno, Juan Maria Torres
Summary: This study demonstrates for the first time that a TSE agent with BSE-like properties can be amplified in cattle inoculated with atypical scrapie brain homogenate.
VETERINARY RESEARCH
(2023)
Article
Biochemistry & Molecular Biology
Mafalda Casanova, Carla Machado, Paula Tavares, Joao Silva, Christine Fast, Anne Balkema-Buschmann, Martin H. Groschup, Leonor Orge
Summary: Portugal was one of the first European countries to report cases of Atypical Scrapie (ASc), and they confirmed through murine bioassays that the ASc strain present in Portuguese sheep corresponds to ASc Nor98.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Microbiology
Tess Gunnels, Ronald A. Shikiya, Taylor C. York, Alyssa J. Block, Jason C. Bartz
Summary: Prion diseases are fatal neurodegenerative disorders affecting various mammalian species, including humans. Prions consist of disease-specific conformations of prion proteins and show heritable phenotypes under controlled transmission conditions. This study reveals that replicating the dominant strain is necessary to suppress the replication of minor substrains, and selective reduction of the dominant strain allows for the emergence of substrains. Preexisting substrains may contribute to prion strain evolution, drug resistance, and interspecies transmission.
Article
Veterinary Sciences
Marina Betancor, Belen Marin, Alicia Otero, Carlos Hedman, Antonio Romero, Tomas Barrio, Eloisa Sevilla, Jean-Yves Douet, Alvina Huor, Juan Jose Badiola, Olivier Andreoletti, Rosa Bolea
Summary: The transmission of atypical scrapie to cattle can lead to the emergence of BSE-like prion seeding activity, even though the infected cattle did not show clinical signs of prion disease.
VETERINARY RESEARCH
(2023)
Article
Veterinary Sciences
Diego Sola, Rody Artigas, Diego R. Mediano, Pilar Zaragoza, Juan Jose Badiola, Inmaculada Martin-Burriel, Cristina Acin
Summary: Prion diseases are fatal neurodegenerative disorders caused by the conversion of cellular prion protein into an abnormal and misfolded isoform. The PRNP gene, which codes for PrP, plays a crucial role in the susceptibility and pathogenesis of prion diseases. This study discovered three novel PRNP polymorphisms in horses and predicted their potential impact on horse prion protein using computational programs. The findings suggest that changes in electrostatic potentials may lead to an increased propensity for amyloid aggregation.
VETERINARY RESEARCH
(2023)
Article
Veterinary Sciences
Eric D. Cassmann, Alexis J. Frese, Kelsey A. Becker, Justin J. Greenlee
Summary: The study found that the E211K genotype of H-BSE in cattle has a shorter incubation period in both homozygous and heterozygous forms, and the type of inoculum does not significantly affect the incubation period.
FRONTIERS IN VETERINARY SCIENCE
(2023)
Article
Biodiversity Conservation
Tolulope I. N. Perrin-Stowe, Yasuko Ishida, Emily E. Terrill, Dan Beetem, Oliver A. Ryder, Jan E. Novakofski, Nohra E. Mateus-Pinilla, Alfred L. Roca
Summary: Variation in the PRNP gene affects susceptibility to Chronic Wasting Disease (CWD), with Pere David's deer having two main PRNP haplotypes associated with reduced vulnerability to CWD. These two haplotypes show similarities to PRNP in other cervids, suggesting long-term balancing selection and potential fitness benefits in maintaining both haplotypes to limit transmission of CWD.
CONSERVATION GENETICS
(2022)
Article
Multidisciplinary Sciences
Hanae Takatsuki, Morikazu Imamura, Tsuyoshi Mori, Ryuichiro Atarashi
Summary: This study demonstrates that RT-QuIC and PMCA can be used to accurately assess therapeutic drug screening for prion diseases, reflecting strain differences.
SCIENTIFIC REPORTS
(2022)
Article
Biochemical Research Methods
Christopher J. Silva, Eric D. Cassmann, Justin J. Greenlee, Melissa L. Erickson-Beltran, Jesus R. Requena
Summary: The transmissibility and progression of scrapie in sheep is heavily influenced by specific amino acid polymorphisms in PrPC. Sheep expressing the K171 polymorphism are partially resistant to scrapie, with a longer incubation period observed. The quantification of the K171 polymorphism in sheep scrapie samples provides important information about the composition of PrPSc.
JOURNAL OF THE AMERICAN SOCIETY FOR MASS SPECTROMETRY
(2023)
Article
Clinical Neurology
Zhongyun Chen, Min Chu, Jing Zhang, Yu Kong, Kexin Xie, Yue Cui, Hong Ye, Li Liu, Junjie Li, Lin Wang, Liyong Wu
Summary: This study aimed to explore the clinical characteristics of sporadic fatal insomnia (sFI), assess its similarities and differences with fatal familial insomnia (FFI), and evaluate the influence of ethnicity on the phenotype of sFI patients. The results showed that sFI had a longer disease duration and a higher proportion of neuropsychiatric symptoms compared to FFI. Differences were also observed in MRI and EEG findings between sFI and FFI patients, especially in those with pathological changes associated with sporadic Creutzfeldt-Jakob disease. Moreover, there were variations in clinical characteristics based on ethnicity in sFI patients.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Virology
James M. Kincheloe, Amy R. Horn-Delzer, Dennis N. Makau, Scott J. Wells
Summary: This study conducted a qualitative risk assessment for CWD transmission to cervid farms based on current scientific understanding and knowledge of farmed cervid operations. The majority of CWD-positive farmed cervid operations in Minnesota and Wisconsin were found to have high transmission risks, but there were also cases with moderate or negligible transmission risks, indicating the need for further investigation to inform effective control measures.
Article
Microbiology
Laura Pirisinu, Michele Angelo Di Bari, Claudia D'Agostino, Ilaria Vanni, Geraldina Riccardi, Stefano Marcon, Gabriele Vaccari, Barbara Chiappini, Sylvie L. L. Benestad, Umberto Agrimi, Romolo Nonno
Summary: Prions are infectious agents that misfold cellular prion protein (PrP(C)) into infectious aggregates (PrPSc), causing fatal neurodegenerative diseases. The transmission of prions depends on the recipient's PrP(C) sequence and the incoming prion strain. Nor98/atypical scrapie (AS) is a less contagious prion disease compared to classical scrapie (CS), and the relationship between AS and CS strains is still unknown. The study found that the M109I polymorphism affects the susceptibility to AS, and AS isolates in different species encoded the same strain. Additionally, minor CS-like strain components were found in AS isolates.
Article
Veterinary Sciences
Soyoun Hwang, Justin J. Greenlee, Eric M. Nicholson
Summary: Chronic wasting disease (CWD) is a fatal transmissible spongiform encephalopathy that is increasing in both wild and farmed cervid populations globally. The lateral transmission of CWD among cervids likely occurs through the shedding of infectious prions into the environment. The use of RT-QuIC assays has shown success in detecting prion seeding activity in fecal samples from clinical and preclinical white-tailed deer, offering a non-invasive and sensitive tool for CWD surveillance.
FRONTIERS IN VETERINARY SCIENCE
(2021)
Article
Biochemistry & Molecular Biology
Oscar Lopez-Perez, David Sanz-Rubio, Adelaida Hernaiz, Marina Betancor, Alicia Otero, Joaquin Castilla, Olivier Andreoletti, Juan Jose Badiola, Pilar Zaragoza, Rosa Bolea, Janne M. Toivonen, Inmaculada Martin-Burriel
Summary: Increased levels of miR-21-5p were found in plasma-derived exosomes from scrapie-infected sheep, while no significant increase was observed in CSF-derived exosomes. However, miR-342-3p, miR-146a-5p, miR-128-3p, and miR-21-5p displayed higher levels in total CSF from scrapie-infected sheep. The presence of overexpressed miRNAs in CSF, along with plasma exosomal miR-21-5p, could aid in the diagnosis of scrapie when the disease is suspected. Additionally, PrPSc was found in most CSF-derived exosomes from clinically affected sheep, potentially facilitating in vivo diagnosis of prion diseases, at least during the clinical stage.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Multidisciplinary Sciences
Alba Marin-Moreno, Juan Carlos Espinosa, Patricia Aguilar-Calvo, Natalia Fernandez-Borges, Jose Luis Pitarch, Lorenzo Gonzalez, Juan Maria Torres
Summary: The protective role of the E/D-163 polymorphism of dog prion protein against prion replication may not be the sole factor for canid prion resistance, as other PrP residues or factors distinct than PrP may participate in this process. Different factors may be required for D-162 sheep PrP to effectively protect sheep against ruminant prions, as observed through inoculation of various prion strains in a transgenic mouse model expressing different PrP variants.
SCIENTIFIC REPORTS
(2021)
Article
Clinical Neurology
Golia Shafiei, Vincent Bazinet, Mahsa Dadar, Ana L. Manera, D. Louis Collins, Alain Dagher, Barbara Borroni, Raquel Sanchez-Valle, Fermin Moreno, Robert Laforce, Caroline Graff, Matthis Synofzik, Daniela Galimberti, James B. Rowe, Mario Masellis, Maria Carmela Tartaglia, Elizabeth Finger, Rik Vandenberghe, Alexandre de Mendonca, Fabrizio Tagliavini, Isabel Santana, Chris Butler, Alex Gerhard, Adrian Danek, Johannes Levin, Markus Otto, Sandro Sorbi, Lize C. Jiskoot, Harro Seelaar, John C. van Swieten, Jonathan D. Rohrer, Bratislav Misic, Simon Ducharme
Summary: The patterns of neurodegeneration in frontotemporal dementia are influenced by the structural network organization. The atrophy patterns in both genetic and sporadic cases mainly affect areas associated with the limbic intrinsic network and insular cytoarchitectonic class. The study findings provide insights into how different pathological entities can lead to the same clinical syndrome.
Article
Clinical Neurology
Steffen Halbgebauer, Samir Abu-Rumeileh, Patrick Oeckl, Petra Steinacker, Francesco Roselli, Diana Wiesner, Angela Mammana, Michael Beekes, Izaro Kortazar-Zubizarreta, Guiomar Perez de Nanclares, Sabina Capellari, Armin Giese, Joaquin Castilla, Albert C. Ludolph, Dana Zakova, Piero Parchi, Markus Otto
Summary: This study investigates the potential application of beta-synuclein as a blood biomarker for early diagnosis and evaluation of synaptic integrity in prion disease.
Article
Pathology
Enric Vidal, Carlos Lopez-Figueroa, Jordi Rodon, Monica Perez, Marco Brustolin, Guillermo Cantero, Victor Guallar, Nuria Izquierdo-Useros, Jorge Carrillo, Julia Blanco, Bonaventura Clotet, Julia Vergara-Alert, Joaquim Segales
Summary: This study chronologically characterized the neuroinvasion and neuropathology of SARS-CoV-2 in a mouse model. The virus was found to enter the brain early through the olfactory mucosa and rapidly spread between neurons, causing acute encephalitis and neuronal damage.
VETERINARY PATHOLOGY
(2022)
Article
Microbiology
Enric Vidal, Judit Burgaya, Lorraine Michelet, Claudia Arrieta-Villegas, Guillermo Cantero, Krystel de Cruz, Jennifer Tambosco, Michelle Di Bari, Romolo Nonno, Maria Laura Boschiroli, Bernat Perez de Val
Summary: A laboratory infection model was established to reproduce Mycobacterium microti infection in bank voles, successfully mimicking the disease phenotype observed in free-ranging voles. The pathogenesis of the infection was characterized, with most voles showing multifocal and diffuse granulomatous lesions in the liver and spleen. Mycobacterial DNA was detected in feces but not in oral swabs, suggesting horizontal transmission between voles.
Article
Clinical Neurology
Hasier Erana, Beatriz San Millan, Carlos M. Diaz-Dominguez, Jorge M. Charco, Rosa Rodriguez, Irene Vieitez, Arrate Pereda, Rosa Yanez, Marivi Geijo, Carmen Navarro, Guiomar Perez de Nanclares, Susana Teijeira, Joaquin Castilla
Summary: Gerstmann-Straussler-Scheinker disease (GSS) is a rare neurodegenerative illness caused by pathogenic alterations in the prion protein (PrP) coding gene, leading to the formation of toxic prions and showing variability in clinical and neuropathological manifestations.
JOURNAL OF NEUROLOGY
(2022)
Article
Neurosciences
Matthias Schmitz, Sezgi Canaslan, Juan Carlos Espinosa, Natalia Fernandez-Borges, Anna Villar-Pique, Franc Llorens, Daniela Varges, Fabian Maass, Juan Maria Torres, Peter Hermann, Inga Zerr
Summary: Biomarkers, specifically neurofilament light chain (NfL), have shown potential as stable and accurate markers for the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD). This study confirms the stability and consistency of CSF and plasma NfL levels in a large cohort of sCJD patients. The newly defined cutoffs for NfL offer good diagnostic accuracies in distinguishing sCJD from control groups. Additionally, the study suggests that NfL could be used as a very early biomarker for sCJD diagnosis.
MOLECULAR NEUROBIOLOGY
(2022)
Article
Microbiology
Alicia Otero, Tomas Barrio, Hasier Erana, Jorge M. Charco, Marina Betancor, Carlos M. Diaz-Dominguez, Belen Marin, Olivier Andreoletti, Juan M. Torres, Qingzhong Kong, Juan J. Badiola, Rosa Bolea, Joaquin Castilla
Summary: In this study, the effect of glycosylation on the characteristics of BSE isolates was assessed using a non-glycosylated human PrPC-expressing mouse model. The findings showed that BSE prions could be propagated in a non-glycosylated human PrPC environment without losing their strain properties. BSE prions transmitted to the transgenic mouse model exhibited neuropathological and biochemical signs compatible with BSE, confirming that the glycosylation of human PrPC is not essential for the propagation of this particular prion strain.
Article
Biochemistry & Molecular Biology
Ilaria Vanni, Floriana Iacobone, Claudia D'Agostino, Matteo Giovannelli, Laura Pirisinu, Hermann Clemens Altmeppen, Joaquin Castilla, Juan Maria Torres, Umberto Agrimi, Romolo Nonno
Summary: The PrPC protein undergoes various endoproteolytic events, resulting in bioactive fragments with physiological functions and implications in neurodegenerative diseases. Lack of methods to identify all PrPC-derived proteoforms has hindered comprehensive investigations. Researchers developed an optimized Western blot assay based on previous knowledge, enabling the identification of the full spectrum of PrPC proteoforms in brain samples.
JOURNAL OF BIOLOGICAL CHEMISTRY
(2023)
Article
Neurosciences
Enric Vidal, Manuel A. Sanchez-Martin, Hasier Erana, Sonia Perez Lazaro, Miguel A. Perez-Castro, Alicia Otero, Jorge M. Charco, Belen Marin, Rafael Lopez-Moreno, Carlos M. Diaz-Dominguez, Marivi Geijo, Montserrat Ordonez, Guillermo Cantero, Michele di Bari, Nuria L. Lorenzo, Laura Pirisinu, Claudia d'Agostino, Juan Maria Torres, Vincent Beringue, Glenn Telling, Juan J. Badiola, Marti Pumarola, Rosa Bolea, Romolo Nonno, Jesus R. Requena, Joaquin Castilla
Summary: Atypical Scrapie is considered a spontaneous idiopathic prion disease in small ruminants, and unlike classical scrapie, its occurrence and control strategies may affect its transmissibility.
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2022)
Article
Ecology
Enric Vidal, Johan Espunyes, Maria Puig Ribas, Cristian Melgarejo, Laura Martino, Lorraine Michelet, Maria Laura Boschiroli, Albert Sanz, Alberto Allepuz, Oscar Cabezon, Bernat Perez de Val
Summary: A study in the Catalan Pyrenees revealed that wild small rodents did not play a role in the epidemiology of M. microti outbreak in the area. The source of this mycobacterium remains unknown, but the movements of wild boars across the French Pyrenees are considered the most likely origin of the outbreak detected in the Iberian Peninsula.
EUROPEAN JOURNAL OF WILDLIFE RESEARCH
(2023)
Article
Agriculture, Dairy & Animal Science
E. Ruiz-Riera, E. Vidal, A. Canturri, A. Lehmbecker, M. Cuvertoret, C. Lopez-Figueroa, W. Baumgaertner, M. Domingo, J. Segales
Summary: This study describes a new pathological condition in pigs, characterized by wasting and vacuolization of the brain. The affected and healthy pigs from eight farms were investigated, and the most consistent lesion observed was neuropil vacuolization in the forebrain. Supplementation with nutritional complexes improved the condition, suggesting a metabolic origin.
Review
Biochemistry & Molecular Biology
Alba Marin-Moreno, Sara Canoyra, Natalia Fernandez-Borges, Juan Carlos Espinosa, Juan Maria Torres
Summary: Neurodegenerative diseases are a major health challenge that modern medicine and advanced societies are facing. Despite years of research, the exact mechanisms leading to neuronal death and effective treatments have not been fully understood. The use of animal models, particularly transgenic mouse models, has provided valuable insights into the pathology of neurodegenerative diseases and the search for potential therapies.
FRONTIERS IN BIOSCIENCE-LANDMARK
(2023)
Article
Microbiology
Carlos Neila-Ibanez, Ester Pintado, Roser Velarde, Xavier Fernandez Aguilar, Enric Vidal, Virginia Aragon, M. Lourdes Abarca
Summary: The study provides information on lesions caused by Streptococcus ruminantium in Pyrenean chamois and domestic sheep, as well as details about the phenotypic biopatterns and antimicrobial resistance of the isolates.
MICROBIOLOGY RESEARCH
(2022)
