Works matching Prion diseases

Results: 5000

A CONTRIBUIÇÃO GENÉTICA NO DESENVOLVIMENTO DE DOENÇAS PRIÔNICAS: REVISÃO SISTEMÁTICA.

Published in:

Revista Foco (Interdisciplinary Studies Journal), 2023, v. 16, n. 6, p. 1, doi. 10.54751/revistafoco.v16n6-096

By:

Bitu de Freitas, Yuri Borges;
Ribeiro Pimentel, Ana Luiza Machado;
Albuquerque Amorim Ayres, Celina Kalena;
Siqueira Bocchi, Giovanna;
Costa Diniz, Henrique;
Rezende Albernaz, Vítor;
Veloso Correia, Davi;
Costa Alves, Bruna

Publication type:

Article

Lidské prionové nemoci v ČR: 22 let zkušeností Národní referenční laboratoře pro diagnostiku lidských prionových chorob.

Published in:

Neurologie Pro Praxi, 2024, v. 25, n. 1, p. 9, doi. 10.36290/neu.2024.002

By:

Jankovská, Nikol;
Matěj, Radoslav

Publication type:

Article

Internal consistency, construct validity, and responsiveness of the MRC Prion Disease Rating Scale.

Published in:

Journal of Patient-Reported Outcomes, 2025, v. 9, n. 1, p. 1, doi. 10.1186/s41687-025-00884-3

By:

Leidy, Leah;
Yarlas, Aaron;
Pulido, Robert S.;
Ludwig, Jessica;
Glisic, Kathleen;
Appleby, Brian S.

Publication type:

Article

A Single Subcutaneous Injection of Cellulose Ethers Administered Long before Infection Confers Sustained Protection against Prion Diseases in Rodents.

Published in:

PLoS Pathogens, 2016, v. 12, n. 12, p. 1, doi. 10.1371/journal.ppat.1006045

By:

Teruya, Kenta;
Oguma, Ayumi;
Nishizawa, Keiko;
Kawata, Maki;
Sakasegawa, Yuji;
Kamitakahara, Hiroshi;
Doh-ura, Katsumi

Publication type:

Article

Increased Abundance of M Cells in the Gut Epithelium Dramatically Enhances Oral Prion Disease Susceptibility.

Published in:

PLoS Pathogens, 2016, v. 12, n. 12, p. 1, doi. 10.1371/journal.ppat.1006075

By:

Donaldson, David S.;
Sehgal, Anuj;
Rios, Daniel;
Williams, Ifor R.;
Mabbott, Neil A.

Publication type:

Article

Humánne priónové ochorenia - „state of the art" 2023.

Published in:

Neurologie Pro Praxi, 2024, v. 25, n. 1, p. 19, doi. 10.36290/neu.2024.005

By:

Kurča, Egon;
Sivák, Štefan;
Skáčik, Pavol

Publication type:

Article

MicroRNA Alterations in the Brain and Body Fluids of Humans and Animal Prion Disease Models: Current Status and Perspectives.

Published in:

Frontiers in Aging Neuroscience, 2018, p. N.PAG, doi. 10.3389/fnagi.2018.00220

By:

Kanata, Eirini;
Thüne, Katrin;
Xanthopoulos, Konstantinos;
Ferrer, Isidre;
Dafou, Dimitra;
Zerr, Inga;
Sklaviadis, Theodoros;
Llorens, Franc

Publication type:

Article

Toll-like receptor 2 confers partial neuroprotection during prion disease.

Published in:

PLoS ONE, 2018, v. 13, n. 12, p. 1, doi. 10.1371/journal.pone.0208559

By:

Carroll, James A.;
Race, Brent;
Williams, Katie;
Chesebro, Bruce

Publication type:

Article

Epigenetic Changes in Prion and Prion-like Neurodegenerative Diseases: Recent Advances, Potential as Biomarkers, and Future Perspectives.

Published in:

2022

By:

Hernaiz, Adelaida;
Toivonen, Janne Markus;
Bolea, Rosa;
Martín-Burriel, Inmaculada

Publication type:

Literature Review

Complement 3<sup>+</sup>-astrocytes are highly abundant in prion diseases, but their abolishment led to an accelerated disease course and early dysregulation of microglia.

Published in:

Acta Neuropathologica Communications, 2019, v. 7, n. 1, p. N.PAG, doi. 10.1186/s40478-019-0735-1

By:

Hartmann, Kristin;
Sepulveda-Falla, Diego;
Rose, Indigo V. L.;
Madore, Charlotte;
Muth, Christiane;
Matschke, Jakob;
Butovsky, Oleg;
Liddelow, Shane;
Glatzel, Markus;
Krasemann, Susanne

Publication type:

Article

Clinical Use of Improved Diagnostic Testing for Detection of Prion Disease.

Published in:

Viruses (1999-4915), 2021, v. 13, n. 5, p. 789, doi. 10.3390/v13050789

By:

Figgie Jr., Mark P.;
Appleby, Brian S.;
Caughey, Byron

Publication type:

Article

Seeded Fibrillation as Molecular Basis of the Species Barrier in Human Prion Diseases.

Published in:

PLoS ONE, 2013, v. 8, n. 8, p. 1, doi. 10.1371/journal.pone.0072623

By:

Luers, Lars;
Bannach, Oliver;
Stöhr, Jan;
Wördehoff, Michael Marius;
Wolff, Martin;
Nagel-Steger, Luitgard;
Riesner, Detlev;
Willbold, Dieter;
Birkmann, Eva

Publication type:

Article

Diagnostic accuracy of cerebrospinal fluid biomarkers in genetic prion diseases.

Published in:

2022

By:

Schmitz, Matthias;
Villar-Piqué, Anna;
Hermann, Peter;
Escaramís, Geòrgia;
Calero, Miguel;
Chen, Cao;
Kruse, Niels;
Cramm, Maria;
Golanska, Ewa;
Sikorska, Beata;
Liberski, Pawel P;
Pocchiari, Maurizio;
Lange, Peter;
Stehmann, Christiane;
Sarros, Shannon;
Martí, Eulàlia;
Baldeiras, Inês;
Santana, Isabel;
Žáková, Dana;
Mitrová, Eva

Publication type:

journal article

Chronic wasting disease: Emerging prions and their potential risk.

Published in:

PLoS Pathogens, 2017, v. 13, n. 11, p. 1, doi. 10.1371/journal.ppat.1006619

By:

Hannaoui, Samia;
Schatzl, Hermann M.;
Gilch, Sabine

Publication type:

Article

TREM2 supports neuronal protection and microglial reactivity without an effect on misfolded protein deposition in chronic neurodegenerative prion disease.

Published in:

Frontiers in Neuroscience, 2025, p. 1, doi. 10.3389/fnins.2025.1525017

By:

Carpanini, Sarah M.;
Bradford, Barry M.;
Alfieri, Alessio;
Piccardo, Pedro;
Monzón-Sandoval, Jimena;
Brown, Deborah;
Boyle, Aileen;
Pokrovskaya, Aleksandra;
Mabbott, Neil A.;
Manson, Jean;
McColl, Barry W.

Publication type:

Article

Downregulation of STAT3 transcription factor reverses synaptotoxic phenotype of reactive astrocytes associated with prion diseases.

Published in:

Acta Neuropathologica Communications, 2025, v. 13, n. 1, p. 1, doi. 10.1186/s40478-025-02028-6

By:

Kushwaha, Rajesh;
Molesworth, Kara;
Makarava, Natallia;
Baskakov, Ilia V.

Publication type:

Article

Validation of the Medical Research Council prion disease rating scale in France.

Published in:

Brain Communications, 2023, v. 5, n. 6, p. 1, doi. 10.1093/braincomms/fcad267

By:

Brandel, Jean-Philippe;
Welaratne, Arlette;
Denouel, Angeline;
Haïk, Stéphane

Publication type:

Article

A case report of genetic prion disease with two different PRNP variants.

Published in:

Molecular Genetics & Genomic Medicine, 2020, v. 8, n. 3, p. 1, doi. 10.1002/mgg3.1134

By:

Piazza, Megan;
Prior, Thomas W.;
Khalsa, Prabhjot S.;
Appleby, Brian

Publication type:

Article

The Role of Unfolded Protein Response and Mitogen-Activated Protein Kinase Signaling in Neurodegenerative Diseases with Special Focus on Prion Diseases.

Published in:

Frontiers in Aging Neuroscience, 2017, v. 9, p. 1, doi. 10.3389/fnagi.2017.00120

By:

Ali Shah, Syed Zahid;
Deming Zhao;
Hussain, Tariq;
Lifeng Yang

Publication type:

Article

Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017.

Published in:

Clinical Epidemiology, 2020, v. 12, p. 1073, doi. 10.2147/CLEP.S274093

By:

Sun, Yu;
Liu, Chih-Ching;
Fan, Ling-Yun;
Huang, Chung-Te;
Chen, Ta-Fu;
Lu, Chien-Jung;
Guo, Wan-Yuo;
Chang, Yang-Chyuan;
Chiu, Ming-Jang

Publication type:

Article

New implications for prion diseases therapy and prophylaxis.

Published in:

Frontiers in Molecular Neuroscience, 2024, p. 1, doi. 10.3389/fnmol.2024.1324702

By:

Fangzhou Liu;
Wenqi Lü;
Ling Liu

Publication type:

Article

Different reactive profiles of calmodulin in the CSF samples of Chinese patients of four types of genetic prion diseases.

Published in:

Frontiers in Molecular Neuroscience, 2024, p. 1, doi. 10.3389/fnmol.2024.1341886

By:

Xiao-Xi Jia;
Chao Hu;
Cao Chen;
Li-Ping Gao;
Dong-Lin Liang;
Wei Zhou;
Run-Dong Cao;
Kang Xiao;
Qi Shi;
Xiao-Ping Dong

Publication type:

Article

In Vivo Longitudinal H MRS Study of Transgenic Mouse Models of Prion Disease in the Hippocampus and Cerebellum at 14.1 T.

Published in:

Neurochemical Research, 2015, v. 40, n. 12, p. 2639, doi. 10.1007/s11064-015-1643-9

By:

Cudalbu, Cristina;
Craveiro, Melanie;
Mlynárik, Vladimir;
Bremer, Juliane;
Aguzzi, Adriano;
Gruetter, Rolf

Publication type:

Article

Prion Disease: A Tale of Folds and Strains.

Published in:

Brain Pathology, 2013, v. 23, n. 3, p. 321, doi. 10.1111/bpa.12045

By:

Kretzschmar, Hans;
Tatzelt, Jörg

Publication type:

Article

When prion disease Isn't suspected: prion disease as the cause of terminal decline in chronic mixed dementia.

Published in:

2023

By:

Krishnamurthy, Sudarshan;
Harrison, William;
Craft, Suzanne;
Lockhart, Samuel N.;
Bateman, James R.

Publication type:

Case Study

Role of Biomarkers for the Diagnosis of Prion Diseases: A Narrative Review.

Published in:

2022

By:

Altuna, Miren;
Ruiz, Iñigo;
Zelaya, María Victoria;
Mendioroz, Maite

Publication type:

Literature Review

Detection limitations of prion seeding activities in blood samples from patients with sporadic prion disease.

Published in:

BMC Neurology, 2024, v. 24, n. 1, p. 1, doi. 10.1186/s12883-024-03590-7

By:

Nonaka, Toshiaki;
Iwasaki, Yasushi;
Horiuchi, Hiroyuki;
Satoh, Katsuya

Publication type:

Article

Neuropathologically directed profiling of PRNP somatic and germline variants in sporadic human prion disease.

Published in:

Acta Neuropathologica, 2024, v. 148, n. 1, p. 1, doi. 10.1007/s00401-024-02774-2

By:

McDonough, Gannon A.;
Cheng, Yuchen;
Morillo, Katherine S.;
Doan, Ryan N.;
Zhou, Zinan;
Kenny, Connor J.;
Foutz, Aaron;
Kim, Chae;
Cohen, Mark L.;
Appleby, Brian S.;
Walsh, Christopher A.;
Safar, Jiri G.;
Huang, August Yue;
Miller, Michael B.

Publication type:

Article

TREM2 expression in the brain and biological fluids in prion diseases.

Published in:

Acta Neuropathologica, 2021, v. 141, n. 6, p. 841, doi. 10.1007/s00401-021-02296-1

By:

Diaz-Lucena, Daniela;
Kruse, Niels;
Thüne, Katrin;
Schmitz, Matthias;
Villar-Piqué, Anna;
da Cunha, Jose Eriton Gomes;
Hermann, Peter;
López-Pérez, Óscar;
Andrés-Benito, Pol;
Ladogana, Anna;
Calero, Miguel;
Vidal, Enric;
Riggert, Joachim;
Pineau, Hailey;
Sim, Valerie;
Zetterberg, Henrik;
Blennow, Kaj;
del Río, Jose Antonio;
Marín-Moreno, Alba;
Espinosa, Juan Carlos

Publication type:

Article

Perspectives on CRISPR Genome Editing to Prevent Prion Diseases in High-Risk Individuals.

Published in:

Biomedicines, 2024, v. 12, n. 8, p. 1725, doi. 10.3390/biomedicines12081725

By:

Medd, Milan M.;
Cao, Qi

Publication type:

Article

The Aetiology of Genetic, Acquired and Sporadic Prion Diseases.

Published in:

Albanian Journal of Agricultural Sciences, 2015, v. 14, n. 1, p. 15

By:

BAJRAMI, NEILA;
BAJRAMI, ZYRI

Publication type:

Article

Downregulation of the Repressor Element 1-Silencing Transcription Factor (REST) Is Associated with Akt-mTOR and Wnt-β-Catenin Signaling in Prion Diseases Models.

Published in:

Frontiers in Molecular Neuroscience, 2017, v. 10, p. 1, doi. 10.3389/fnmol.2017.00128

By:

Zhiqi Song;
Shah, Syed Z. A.;
Wei Yang;
Haodi Dong;
Lifeng Yang;
Xiangmei Zhou;
Deming Zhao

Publication type:

Article

Gene regulatory network analysis supports inflammation as a key neurodegeneration process in prion disease.

Published in:

BMC Systems Biology, 2012, v. 6, n. 1, p. 1, doi. 10.1186/1752-0509-6-132

By:

Crespo, Isaac;
Roomp, Kirsten;
Jurkowski, Wiktor;
Kitano, Hiroaki;
del Sol, Antonio

Publication type:

Article

A Preliminary Study for the Determination of Prion Disease for Farm Animals in Ethiopia.

Published in:

Journal of Animal Production / Hayvansal Üretim, 2020, v. 61, n. 1, p. 17, doi. 10.29185/hayuretim.583573

By:

TEFERDEGN, Eden Yitna;
ÜN, Cemal;
TAŞKIN, Turğay

Publication type:

Article

Plasma total prion protein as a potential biomarker for neurodegenerative dementia: diagnostic accuracy in the spectrum of prion diseases.

Published in:

Neuropathology & Applied Neurobiology, 2020, v. 46, n. 3, p. 240, doi. 10.1111/nan.12573

By:

Llorens, F.;
Villar‐Piqué, A.;
Schmitz, M.;
Diaz‐Lucena, D.;
Wohlhage, M.;
Hermann, P.;
Goebel, S.;
Schmidt, I.;
Glatzel, M.;
Hauw, J.‐J.;
Sikorska, B.;
Liberski, P. P.;
Riggert, J.;
Ferrer, I.;
Zerr, I.

Publication type:

Article

Dysfunction of the PI3 K- Akt- GSK-3 pathway is a common feature in cell culture and in vivo models of prion disease.

Published in:

Neuropathology & Applied Neurobiology, 2014, v. 40, n. 3, p. 311, doi. 10.1111/nan.12066

By:

Simon, D.;
Herva, M. E.;
Benitez, M. J.;
Garrido, J. J.;
Rojo, A. I.;
Cuadrado, A.;
Torres, J. M.;
Wandosell, F.

Publication type:

Article

Phenotypic variability in human prion diseases.

Published in:

Neuropathology & Applied Neurobiology, 2005, v. 31, n. 6, p. 565, doi. 10.1111/j.1365-2990.2005.00697.x

By:

Ironside, J. W.;
Ritchie, D. L.;
Head, M. W.

Publication type:

Article

Ultrastructural pathology of prion diseases revisited: brain biopsy studies.

Published in:

Neuropathology & Applied Neurobiology, 2005, v. 31, n. 1, p. 88, doi. 10.1111/j.1365-2990.2004.00595.x

By:

Liberski, P. P.;
Streichenberger, N.;
Giraud, P.;
Soutrenon, M.;
Meyronnet, D.;
Sikorska, B.;
Kopp, N.

Publication type:

Article

Phenotypic variability in the brains of a family with a prion disease characterized by a 144-base pair insertion in the prion protein gene.

Published in:

Neuropathology & Applied Neurobiology, 2003, v. 29, n. 2, p. 98, doi. 10.1046/j.1365-2990.2003.00423.x

By:

King, A.;
Doey, L.;
Rossor, M.;
Mead, S.;
Collinge, J.;
Lantos, P.

Publication type:

Article

Differential expression of transforming growth factor-β isoforms in human prion diseases.

Published in:

Neuropathology & Applied Neurobiology, 1998, v. 24, n. 4, p. 284, doi. 10.1046/j.1365-2990.1998.00117.x

By:

Tashiro;
Doh-ura;
Iwaki;
Tashiro

Publication type:

Article

Review on prion diseases in animals with emphasis to Bovine Spongiform Encephalopathy.

Published in:

Veterinary World, 2012, v. 5, n. 7, p. 443, doi. 10.5455/vetworld.2012.443-448

By:

Londhe, Mukund S.;
Mahajan, Nand K.;
Gupta, Rajender P.;
Londhe, Renu M.

Publication type:

Article

Innate Immune Tolerance in Microglia Does Not Impact on Central Nervous System Prion Disease.

Published in:

Frontiers in Cellular Neuroscience, 2022, v. 16, p. 1, doi. 10.3389/fncel.2022.918883

By:

Pal, Reiss;
Bradford, Barry M.;
Mabbott, Neil A.

Publication type:

Article

The Evolution of Experimental Rodent Models for Prion Diseases.

Published in:

Journal of Neurochemistry, 2025, v. 169, n. 3, p. 1, doi. 10.1111/jnc.70039

By:

DeFranco, Joseph P.;
Telling, Glenn C.

Publication type:

Article

Strain‐dependent role of copper in prion disease through binding to histidine residues in the N‐terminal domain of prion protein.

Published in:

Journal of Neurochemistry, 2023, v. 167, n. 3, p. 394, doi. 10.1111/jnc.15971

By:

Hara, Hideyuki;
Miyata, Hironori;
Chida, Junji;
Sakaguchi, Suehiro

Publication type:

Article

Cellular prion protein neuroprotective function: implications in prion diseases.

Published in:

Journal of Molecular Medicine, 2005, v. 83, n. 1, p. 3, doi. 10.1007/s00109-004-0605-5

By:

Roucou, Xavier;
LeBlanc, Andréa C.

Publication type:

Article

The Role of PET Imaging in Patients with Prion Disease: A Literature Review.

Published in:

Molecular Imaging & Biology, 2024, v. 26, n. 2, p. 195, doi. 10.1007/s11307-024-01895-0

By:

Mattoli, Maria Vittoria;
Giancipoli, Romina Grazia;
Cocciolillo, Fabrizio;
Calcagni, Maria Lucia;
Taralli, Silvia

Publication type:

Article

Prion disease in Indigenous Australians.

Published in:

Internal Medicine Journal, 2021, v. 51, n. 7, p. 1101, doi. 10.1111/imj.14835

By:

Panegyres, Peter K.;
Stehmann, Christiane;
Klug, Genevieve M.;
Masters, Colin L.;
Collins, Steven

Publication type:

Article

Carnosic Acid and Carnosol Display Antioxidant and Anti-Prion Properties in In Vitro and Cell-Free Models of Prion Diseases.

Published in:

Antioxidants, 2022, v. 11, n. 4, p. N.PAG, doi. 10.3390/antiox11040726

By:

Karagianni, Korina;
Pettas, Spyros;
Kanata, Eirini;
Lioulia, Elisavet;
Thune, Katrin;
Schmitz, Matthias;
Tsamesidis, Ioannis;
Lymperaki, Evgenia;
Xanthopoulos, Konstantinos;
Sklaviadis, Theodoros;
Dafou, Dimitra

Publication type:

Article

Longitudinal microbiome investigation throughout prion disease course reveals pre- and symptomatic compositional perturbations linked to short-chain fatty acid metabolism and cognitive impairment in mice.

Published in:

Frontiers in Microbiology, 2024, p. 1, doi. 10.3389/fmicb.2024.1412765

By:

Losa, Marco;
Morsy, Yasser;
Emmenegger, Marc;
Manz, Salomon M.;
Schwarz, Petra;
Aguzzi, Adriano;
Scharl, Michael

Publication type:

Article

Regulation of MicroRNAs-Mediated Autophagic Flux: A New Regulatory Avenue for Neurodegenerative Diseases With Focus on Prion Diseases.

Published in:

Frontiers in Aging Neuroscience, 2018, p. 1, doi. 10.3389/fnagi.2018.00139

By:

Shah, Syed Zahid Ali;
Deming Zhao;
Hussain, Tariq;
Sabir, Naveed;
Lifeng Yang

Publication type:

Article