Article
Biochemistry & Molecular Biology
Stefanie-Elisabeth Heumueller, Annika C. Hornberger, Alina S. Hebestreit, Andre Hossinger, Ina M. Vorberg
Summary: This translation provides an overview of the characteristics of prion invasion, replication, and dissemination, highlighting the unconventional nature of prions and their reliance on host cell machinery.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
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
Neurosciences
Hasier Erana, Carlos M. Diaz-Dominguez, Jorge M. Charco, Enric Vidal, Ezequiel Gonzalez-Miranda, Miguel A. Perez-Castro, Patricia Pineiro, Rafael Lopez-Moreno, Cristina Sampedro-Torres-Quevedo, Leire Fernandez-Veiga, Juan Tasis-Galarza, Nuria L. Lorenzo, Aileen Santini-Santiago, Melisa Lazaro, Sandra Garcia-Martinez, Nuno Goncalves-Anjo, Maitena San-Juan-Ansoleaga, Josu Galarza-Ahumada, Eva Fernandez-Munoz, Samanta Giler, Mikel Valle, Glenn C. Telling, Marivi Geijo, Jesus R. Requena, Joaquin Castilla
Summary: This study presents a method that consistently induces spontaneous misfolding of recombinant PrP into prions within hours, providing unprecedented opportunities to investigate the mechanisms underlying sporadic prion diseases.
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2023)
Article
Biochemistry & Molecular Biology
Nikita Admane, Ankit Srivastava, Salma Jamal, Ritika Sharma, Bishwajit Kundu, Abhinav Grover
Summary: This study examined the role of gallate group in modulating the aggregation and toxicity of human prion protein (HuPrP) using two green tea catechins. The results showed that the gallate group significantly reduced the aggregation and toxicity of HuPrP and had potential fibril disaggregating properties. This finding provides a new structural motif for designing therapeutics against prion diseases and other neurodegenerative disorders.
INTERNATIONAL JOURNAL OF BIOLOGICAL MACROMOLECULES
(2022)
Article
Multidisciplinary Sciences
Maximo Sanz-Hernandez, Joseph D. Barritt, Jens Sobek, Simone Hornemann, Adriano Aguzzi, Alfonso De Simone
Summary: The T183A variant of human PrP significantly enhances aggregation propensity, leading to amyloid formation under physiological conditions by the sole C-terminal domain of the protein. The study identified the structural characteristics of the misfolded intermediate promoting aggregation of T183A huPrP and the interactions preventing the population of this species in the wild-type protein, supporting the use of POM antibodies to suppress the aggregation of this amyloidogenic mutant.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Review
Virology
Jiyan Ma, Jingjing Zhang, Runchuan Yan
Summary: The generation of recombinant prions has provided valuable insights into the characteristics and effects of prions. Recombinant prions can exist in various misfolded conformations and have different outcomes when inoculated into wild-type animals. The ability to seed alone is not sufficient to determine prion activity, as authentic prions need to be both heritable and pathogenic. Research on recombinant prions is important for understanding the pathogenesis of prion diseases and the role of misfolded proteins in other neurodegenerative disorders.
Article
Biochemistry & Molecular Biology
Puspita Halder, Pralay Mitra
Summary: Human familial prion diseases are associated with different single-point mutants of the gene coding for prion protein, with 12 identified pathogenic mutants of human prion protein. These mutants exhibit unfolding of the helical structures, extension and generation of the b-sheet structures, and highly exposed hydrophobic surfaces, possibly leading to the production of aggregate/fibril structures of the prion protein.
JOURNAL OF BIOMOLECULAR STRUCTURE & DYNAMICS
(2022)
Article
Infectious Diseases
Hong Li, Haoju Pan, Le Xu, Suya Li, Shiyuan Li, Si Chen, Churiga Man, Li Du, Qiaoling Chen, Jianhua Xiao, Hongbin Wang, Fengyang Wang, Hongyan Gao
Summary: Based on prion occurrence records and high-resolution environmental layers, this study conducted a risk assessment of classical scrapie in China using a maximum entropy model. The important factors influencing the occurrence of classical scrapie were identified, and the risk map showed potential high-risk areas mainly located in Northeast, North, and Northwest China. This study provides a valuable reference for the prevention of classical scrapie in China.
TRANSBOUNDARY AND EMERGING DISEASES
(2023)
Review
Medicine, General & Internal
Nikol Jankovska, Robert Rusina, Magdalena Bruzova, Eva Parobkova, Tomas Olejar, Radoslav Matej
Summary: Human prion disorders, caused by misfolded prion protein aggregation, are monitored actively in most countries, with the Czech Republic centralizing data at the National surveillance center. Over the past 20 years, a total of 305 cases of sporadic and genetic TSEs have been confirmed, including 8 rare cases of GSS. Additionally, no negative impact on TSEs surveillance has been observed in the Czech Republic, even during the COVID-19 pandemic.
Article
Chemistry, Multidisciplinary
Preeti Rana Sirohi, Anchala Kumari, Nikita Admane, Pallavi Somvanshi, Abhinav Grover
Summary: The study demonstrates the potential of polydatin in inhibiting prion fibrillogenesis by stabilizing the amyloidogenic region of prion protein. This finding highlights the importance of polydatin in combating prion pathogenesis, suggesting it could be a structural scaffold for developing novel therapeutic agents targeting amyloidogenic transition in prions.
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
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
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
Clinical Neurology
Melanie Alpaugh, Francesca Cicchetti
Summary: Research suggests that Huntington's disease shares similarities with prion disorders and may also have sporadic forms, indicating potential common mechanisms governing these neurodegenerative disorders.
JOURNAL OF NEUROLOGY
(2021)
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)