Article
Microbiology
Alyssa J. Block, Ronald A. Shikiya, Thomas E. Eckland, Anthony E. Kincaid, Ryan W. Walters, Jiyan Ma, Jason C. Bartz
Summary: Murine synthetic prions can efficiently establish infection in hamsters and quickly adapt, showing similar clinical and biochemical properties to known prion strains. This model could be valuable for exploring the structure-function relationships between PrPSc and PrP(C) from different species.
Article
Virology
Chih-Yen King
Summary: This passage discusses the replication mechanism of prions and the emergence of variants and strains. It is suggested that the heat shock protein Hsp104 can restrict strain variation, leading to the possibility that some transmutable variants may have been mistaken as faithful-propagating. By altering the strength of Hsp104 in yeast, it is found that most variants are faithful-propagating, with only a few capable of changing to limited structural types.
Article
Virology
Sarah C. Miller, Andrea K. Wegrzynowicz, Sierra J. Cole, Rachel E. Hayward, Samantha J. Ganser, Justin K. Hines
Summary: Yeast prions are protein-based transmissible elements that can be propagated through the chaperone protein network. In this study, we found that the JDP-Hsp70 system is required for the propagation of synthetic prions and that Sis1 acts broadly on amyloids, rather than interacting specifically with individual prion-forming proteins.
Article
Construction & Building Technology
Minggang Shang, Hongxia Qiao, Zhongmao He, Yunsheng Zhang, Qiong Feng, Cuizhen Xue, Yu Zhang, Shengtian Zhai, Jinpen Wang, Yuehui Han
Summary: This study investigates the application of waste incineration tailings as lightweight aggregates in specified density concrete, focusing on the influence of dosage and strain rate. The results show that the addition of tailings improves the impact resistance and dynamic mechanical properties of the concrete. It also enhances the degree of fragmentation, peak stress, and peak strain. The proposed dynamic constitutive equation provides good applicability for numerical calculations. Waste incineration tailings can be a sustainable and cost-effective substitute for some cement-based materials.
CONSTRUCTION AND BUILDING MATERIALS
(2023)
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
Chemistry, Multidisciplinary
Martin Kretschmer, Rafael Cena-Diez, Cosmin Butnarasu, Valentin Silveira, Illia Dobryden, Sonja Visentin, Per Berglund, Anders Sonnerborg, Oliver Lieleg, Thomas Crouzier, Hongji Yan
Summary: Mucus is a self-healing gel that provides protection by binding to and removing viruses, with bovine submaxillary mucins forming gels that exhibit lubricating properties and reduce HIV-1 and HSV-2 infectivity.
Article
Multidisciplinary Sciences
Karla A. Schwenke, Joo-Hee Waelzlein, Agnieszka Bauer, Achim Thomzig, Michael Beekes
Summary: Animal models have played an important role in prion research, but ethical concerns, cost, and labor-saving aspects have led to the search for alternatives in vitro. Bank voles show unique susceptibility to prions from various species, making them a valuable cell model for studying and comparing different prion strains with diverse species backgrounds.
SCIENTIFIC REPORTS
(2022)
Article
Chemistry, Physical
Xiumei Dong, Caixia Mao, Libing Qian, Yonghong Hu, Li Xue, Haiming Huang
Summary: A new stable structure of monolayer h-CSe has been predicted using the first-principles method, and its geometrical, electronic, and optical properties have been investigated. The results show that monolayer h-CSe possesses a tunable bandgap, sensitive to in-plane strain and layer number, and exhibits excellent optical properties.
PHYSICAL CHEMISTRY CHEMICAL PHYSICS
(2023)
Article
Chemistry, Physical
Xiumei Dong, Caixia Mao, Libing Qian, Yonghong Hu, Li Xue, Haiming Huang
Summary: A new structure of monolayer h-CSe with excellent stability was predicted. It is found to be an indirect semiconductor with a tunable bandgap by altering the strain and layer number. It also exhibits strong optical properties.
PHYSICAL CHEMISTRY CHEMICAL PHYSICS
(2023)
Article
Mechanics
Yanru Mu, Yuan Jin, Han Ji, Weiwei Wang, Sijia Zou, Chao Zhang, Jianke Du
Summary: In this study, a hierarchical cellular structure based on TPMS was proposed and its mechanical performance was analyzed through experiments and numerical simulations. The results showed that the structure exhibited good energy absorption characteristics and relatively low first peak stresses.
COMPOSITE STRUCTURES
(2023)
Article
Computer Science, Artificial Intelligence
Shogo Hayashi, Junya Honda, Hisashi Kashima
Summary: Bayesian optimization (BO) is a method for optimizing expensive and difficult-to-evaluate black-box functions, but specifying values for all input variables can be challenging. This paper introduces a new approach called Gaussian process bandit problem, BO with partially specified queries (BOPSQ), which allows for only some input variables to be specified. Algorithms based on posterior sampling are proposed for known and unknown input distributions, with demonstrated effectiveness on test functions and real-world datasets.
Article
Chemistry, Physical
Huabing Shu
Summary: Mixing silicon and carbon to form graphene-like silicon carbide SiC2 provides a novel way to open a desirable gap in semimetallic silicene and graphene. The pristine SiC2 exhibits strong dynamical stabilities and behaves as a direct semiconductor with a moderate gap that can be modulated by biaxial strain. SiC2 shows superior optical absorption for near-infrared and visible light, with a large exciton binding energy, and can have optical absorption peaks significantly red-shifted under applied biaxial strain. These findings suggest great potential for nanoelectronics and optoelectronics applications.
APPLIED SURFACE SCIENCE
(2021)
Article
Chemistry, Physical
Tuan V. Vu, Huynh Phuc, Chuong Nguyen, Vo T. T. Vi, A. Kartamyshev, Nguyen N. Hieu
Summary: In this study, we investigate the stability, electronic properties, and transport characteristics of four asymmetric monolayers XWGeN2 using ab initio density functional theory. The results show that all four monolayers are stable indirect semiconductors, and exhibit significant spin splitting and Rashba-type spin splitting. TeWGeN2 has the largest Rashba energy, which makes it potentially valuable for applications in nanoelectronic devices.
PHYSICAL CHEMISTRY CHEMICAL PHYSICS
(2022)
Article
Construction & Building Technology
Meng Chen, Guangquan Wang, Tong Zhang, Mingyang Ma, Shubin Tang, Yao Zhang
Summary: This study investigates the feasibility of partially replacing polyvinyl alcohol (PVA) fibers with recycled tire steel (RTS) fibers in engineered cementitious composites (ECC) to improve their dynamic mechanical behavior and make use of recycled waste tires. Laboratory tests were conducted to evaluate the workability, elastic modulus, uniaxial tensile behavior, and dynamic splitting tensile behavior of ECC containing PVA and RTS fibers. The results show that replacing 0.25% PVA fibers with RTS fibers decreases the tensile strength by 0.85 MPa but significantly increases the first-crack strength. RTS fibers also contribute to maintaining specimen integrity under dynamic loading, resulting in increased dynamic splitting strength and dissipation energy compared to using PVA fibers. The predictions of the dynamic increase factor with strain rate match well with the experimental results.
CONSTRUCTION AND BUILDING MATERIALS
(2023)
Article
Polymer Science
Chuanhui Gao, Rixuan Wang, Yue Feng, Picheng Chen, Yanqing Wang, Yuetao Liu, Lishui Sun
Summary: In this paper, a new strategy to enhance the strength and tear resistance of deep eutectic gel is presented by inducing PVA crystallization with a deep eutectic solvent (DESD) as the polymerizable component. The fully non-covalent cross-linked structure of the gel enables excellent self healing and recycling ability. This strategy provides a new idea for the preparation of high strength ionic conductive gel.
EUROPEAN POLYMER JOURNAL
(2023)
Review
Cell Biology
Elisa Uliassi, Lea Nikolic, Maria Laura Bolognesi, Giuseppe Legname
Summary: This article reviews the major advances in prion drug discovery, including the identification of anti-prion small molecules through screening methods, as well as the discovery of novel compounds with expanded anti-prion profiles through multi-target-directed ligand (MTDLs) strategies and theranostics.
CELL AND TISSUE RESEARCH
(2023)
Article
Medicine, General & Internal
Neil Watson, Peter Hermann, Anna Ladogana, Angeline Denouel, Simone Baiardi, Elisa Colaizzo, Giorgio Giaccone, Markus Glatzel, Alison J. E. Green, Stephane Haik, Daniele Imperiale, Janet MacKenzie, Fabio Moda, Colin Smith, David Summers, Dorina Tiple, Luana Vaianella, Gianluigi Zanusso, Maurizio Pocchiari, Inga Zerr, Piero Parchi, Jean-Philippe Brandel, Suvankar Pal
Summary: This diagnostic study demonstrated significantly improved sensitivity of revised sCJD diagnostic criteria with unaltered specificity. The revision has enhanced diagnostic accuracy for clinical care and surveillance.
Article
Clinical Neurology
Paola Saveri, Stefania Magri, Emanuela Maderna, Francesca Balistreri, Raffaella Lombardi, Claudia Ciano, Fabio Moda, Barbara Garavaglia, Chiara Reale, Giuseppe Lauria Pinter, Franco Taroni, Davide Pareyson, Chiara Pisciotta
Summary: We describe a family with CMT2 caused by a homozygous DNAJB2 mutation and provide insights into the pathomechanisms. The mutation leads to severe muscle weakness, loss of movement, and reduced sensation in the lower limbs. Patients also exhibit severe hearing loss and one patient has Parkinson's disease. The study reveals reduced levels of DNAJB2 mRNA and protein, as well as the presence of phospho-alpha-synuclein deposits and TDP-43 accumulation in the patients.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Article
Neurosciences
Marco Zattoni, Marika Mearelli, Silvia Vanni, Arianna Colini Baldeschi, Thanh Hoa Tran, Chiara Ferracin, Marcella Catania, Fabio Moda, Giuseppe Di Fede, Giorgio Giaccone, Fabrizio Tagliavini, Gianluigi Zanusso, James W. Ironside, Isidre Ferrer, Giuseppe Legname
Summary: This study investigated the differential expression of serpin superfamily members in neurodegenerative diseases. The results showed dysregulation of SERPINB1, SERPINB6, SERPING1, SERPINH1, and SERPINI1 in sCJD individuals compared to controls, while only SERPINB1 was upregulated in AD patients. Additionally, SerpinA3n transcript and protein were upregulated in a mouse model of AD. These findings suggest that SERPINA3/SerpinA3n may be a potential therapeutic target for the treatment of prion and prion-like neurodegenerative diseases.
MOLECULAR NEUROBIOLOGY
(2022)
Correction
Clinical Neurology
Anna Rita Giovagnoli, Giuseppe Di Fede, Giacomina Rossi, Fabio Moda, Marina Grisoli, Orso Bugiani
NEUROLOGICAL SCIENCES
(2022)
Article
Geriatrics & Gerontology
Federico Angelo Cazzaniga, Edoardo Bistaffa, Chiara Maria Giulia De Luca, Sara Maria Portaleone, Marcella Catania, Veronica Redaelli, Irene Tramacere, Giuseppe Bufano, Martina Rossi, Paola Caroppo, Anna Rita Giovagnoli, Pietro Tiraboschi, Giuseppe Di Fede, Roberto Eleopra, Grazia Devigili, Antonio Emanuele Elia, Roberto Cilia, Michele Fiorini, Matilde Bongianni, Giulia Salzano, Luigi Celauro, Federico Giuseppe Quarta, Angela Mammana, Giuseppe Legname, Fabrizio Tagliavini, Piero Parchi, Gianluigi Zanusso, Giorgio Giaccone, Fabio Moda
Summary: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder caused by the conformational conversion of the prion protein. The PMCA technique can detect PrPSc in the olfactory mucosa and estimate its concentration. Optimizing PMCA is important for diagnosis and selection of patients for clinical trials.
FRONTIERS IN AGING NEUROSCIENCE
(2022)
Review
Clinical Neurology
Giovanni Bellomo, Chiara Maria Giulia De Luca, Federico Paolini Paoletti, Lorenzo Gaetani, Fabio Moda, Lucilla Parnetti
Summary: Parkinson disease (PD) is a common neurodegenerative disease associated with misfolding and aggregation of alpha-synuclein. Seed amplification assays (SAAs) can detect misfolded alpha-synuclein in patients with PD and are promising diagnostic biomarkers. However, standardization issues need to be addressed.
Article
Chemistry, Medicinal
Matteo Staderini, Silvia Vanni, Arianna Colini Baldeschi, Gabriele Giachin, Marco Zattoni, Luigi Celauro, Chiara Ferracin, Edoardo Bistaffa, Fabio Moda, Daniel Perez, Ana Martinez, M. Antonia Martin, Olmo Martin-Camara, Angel Cores, Giulia Bianchini, Robert Kammerer, J. Carlos Menendez, Giuseppe Legname, Maria Laura Bolognesi
Summary: This study designed novel carbazole derivatives as pharmacological chaperones for prion diseases. These derivatives showed anti-prion activity and could detect prion protein aggregates through fluorescent staining. The compounds exhibited activity against RML-infected cell lines and have potential applications in the treatment of prion diseases.
EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY
(2023)
Review
Biochemistry & Molecular Biology
Giuseppe Legname
Summary: In mammals, the prion protein (PrPC) is a common protein involved in various functions in the central nervous system. It also plays a central role in prion diseases, a group of fatal neurodegenerative disorders. The protein can convert into an infectious form called prion and its unique structure, including octarepeat sequences and copper binding sites, may modulate prion conversion and replication.
Letter
Clinical Neurology
Miryam Cannizzaro, Valentina Pieri, Davide Curti, Mor Gueye, Giulia Rugarli, Giovanni Napoli, Rosalinda Cardamone, Andrea Falini, Daniela Perani, Marco Zucconi, Francesca Casoni, Giorgio Giaccone, Fabio Moda, Giuseppe Di Fede, Giuseppe Magnani, Francesca Caso, Massimo Filippi
JOURNAL OF NEUROLOGY
(2023)
Correction
Geriatrics & Gerontology
Federico Angelo Cazzaniga, Edoardo Bistaffa, Chiara Maria Giulia De Luca, Sara Maria Portaleone, Marcella Catania, Veronica Redaelli, Irene Tramacere, Giuseppe Bufano, Martina Rossi, Paola Caroppo, Anna Rita Giovagnoli, Pietro Tiraboschi, Giuseppe Di Fede, Roberto Eleopra, Grazia Devigili, Antonio Emanuele Elia, Roberto Cilia, Michele Fiorini, Matilde Bongianni, Giulia Salzano, Luigi Celauro, Federico Giuseppe Quarta, Angela Mammana, Giuseppe Legname, Fabrizio Tagliavini, Piero Parchi, Gianluigi Zanusso, Giorgio Giaccone, Fabio Moda
FRONTIERS IN AGING NEUROSCIENCE
(2023)
Article
Clinical Neurology
Sandra Pritzkow, Frank Ramirez, Adam Lyon, Paul E. Schulz, Brian Appleby, Fabio Moda, Santiago Ramirez, Silvio Notari, Pierluigi Gambetti, Claudio Soto
Summary: This study evaluated the presence of prions in the urine of patients with the major subtypes of sCJD. It found that 36% of patients tested positive for prions in their urine, indicating a potential risk for inter-individual transmission of infectious prions.
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY
(2023)
Article
Biochemistry & Molecular Biology
Luigi Celauro, Marco Zattoni, Giuseppe Legname
Summary: Cellular prion protein plays a significant role in neurodegenerative diseases and can be taken up by neighboring cells. The intercellular routing of prion proteins involves the release through vesicles and nanotubes.
RECEPTOR ENDOCYTOSIS AND SIGNALLING IN HEALTH AND DISEASE, PT B
(2023)
Article
Chemistry, Multidisciplinary
Luigi Russo, Giulia Salzano, Andrea Corvino, Edoardo Bistaffa, Fabio Moda, Luigi Celauro, Gianluca D'Abrosca, Carla Isernia, Danilo Milardi, Gabriele Giachin, Gaetano Malgieri, Giuseppe Legname, Roberto Fattorusso
Summary: This study investigates the mechanism of thermal unfolding of human prion protein and reveals that the transient electrostatic interactions between the N- and C-terminal domains play a key role in regulating the folding process. The study also provides a clear molecular description of the initial phases of prion misfolding using fluorescence fibrillization assays.
