Results 41 to 50 of about 5,869 (184)

Detection of bovine spongiform encephalopathy, ovine scrapie prion-related protein (PrPSc) and normal PrPc by monoclonal antibodies raised to copper-refolded prion protein [PDF]

Biochemical Journal, 2003
Prion-related protein (PrP) is a glycosylphosphatidylinositol-linked cell-surface protein expressed by a wide variety of cells, including those of the nervous system and the immune system. Several functions of normal cellular PrP (PrPc) have been proposed that may be associated with the capacity of this protein to bind copper.
Alana M, Thackray, Jean-Yves, Madec, Edmond, Wong, Robert, Morgan-Warren, David R, Brown, Thierry, Baron, Raymond, Bujdoso   +6 more
openaire   +2 more sources

Challenges and Advances in Antemortem Diagnosis of Human Transmissible Spongiform Encephalopathies

Frontiers in Bioengineering and Biotechnology, 2020
Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, arise from the structural conversion of the monomeric, cellular prion protein (PrPC) into its multimeric scrapie form (PrPSc).
Lucas M. Ascari, Stephanie C. Rocha, Priscila B. Gonçalves, Tuane C. R. G. Vieira, Yraima Cordeiro   +4 more
doaj   +1 more source

Enhanced Sensitivity of a Modified Quaking‐Induced Conversion Diagnostic Test for the Broad Detection of Sporadic and Inherited Prion Diseases: A Retrospective Study

Annals of Neurology, EarlyView.
Objective Quaking‐induced conversion (QuIC) tests, which detect prion‐seeding activity in cerebrospinal fluid (CSF), have markedly advanced the antemortem diagnosis of prion diseases such as Creutzfeldt‐Jakob disease (CJD). These tests provide high diagnostic accuracy and enable timely differentiation from other rapidly progressive neurodegenerative ...
Jennifer Myskiw, Rebecca Fox, Dominic M.S. Kielich, Lise Lamoureux, Melanie Leonhardt, Olivia Nykvist, Jessy A. Slota, Kristen Avery, Clark Phillipson, Kathy Frost, Sharon Simon, Brian S. Appleby, Ben A. Bailey‐Elkin, Stephanie A. Booth   +13 more
wiley   +1 more source

PrP(Sc)-specific antibodies with the ability to immunodetect prion oligomers.

PLoS ONE, 2011
The development of antibodies with binding capacity towards soluble oligomeric forms of PrPSc recognised in the aggregation process in early stage of the disease would be of paramount importance in diagnosing prion diseases before extensive ...
Mourad Tayebi, Daryl Rhys Jones, William Alexander Taylor, Benjamin Frederick Stileman, Charlotte Chapman, Deming Zhao, Monique David   +6 more
doaj   +1 more source

NUAK2 Inhibition Enhances Macromolecular Drug Delivery in a 3D Fibrotic Model of the Pancreatic Tumor Microenvironment

Advanced NanoBiomed Research, EarlyView.
Using a 3D cell culture model of the fibrotic barrier in pancreatic cancer, this work shows that inhibiting NUAK2 kinase in pancreatic stellate cells enhances macromolecular drug delivery. Mechanistically, NUAK2 inhibition disrupted actin stress fiber assembly to downregulate collagen I expression, thus enhancing macromolecular permeability.
Misaki Nakamura, Hiroyoshi Y. Tanaka, Mayu Ohira, Haruko Ohta‐Okano, Reika Nakamura, Yu Seno, Saaya Yara, Sakura Fujita, Daichi Shibata, Masaya Yamamoto, Shinichi Toyooka, Kensuke Osada, Horacio Cabral, Atsushi Masamune, Mitsunobu R. Kano   +14 more
wiley   +1 more source

Phosphatidylinositol-glycan-phospholipase D is involved in neurodegeneration in prion disease. [PDF]

PLoS ONE, 2015
PrPSc is formed from a normal glycosylphosphatidylinositol (GPI)-anchored prion protein (PrPC) by a posttranslational modification. Most GPI-anchored proteins have been shown to be cleaved by GPI phospholipases.
Jae-Kwang Jin, Byungki Jang, Hyoung Tae Jin, Eun-Kyoung Choi, Cha-Gyun Jung, Hiroyasu Akatsu, Jae-Il Kim, Richard I Carp, Yong-Sun Kim   +8 more
doaj   +1 more source

Rapid generation of prion disease models using AAV‐delivered PrP variants in knockout mice

Brain Pathology, EarlyView.
We developed a rapid AAV‐based system to generate prion disease models in weeks rather than months. Following systemic AAV9P31 delivery of modified PrP to knockout mice, we achieved brain‐wide expression and successful propagation of both classical (RML) and atypical (GSS‐A117V) prion strains.
Maitena San‐Juan‐Ansoleaga, Eva Fernández‐Muñoz, Jorge M. Charco, Enric Vidal, Diego Herrero‐Martínez, Josu Galarza‐Ahumada, Cristina Sampedro‐Torres‐Quevedo, Samanta Giler, Mariví Geijo, Gloria González‐Aseguinolaza, Hasier Eraña, Joaquín Castilla   +11 more
wiley   +1 more source

The L108I polymorphism in mouse prion protein drives spontaneous disease and enhances transmission of atypical and classical prion strains

Brain Pathology, EarlyView.
A single amino acid change (L108I) combined with PrP overexpression drives spontaneous atypical prion formation in mice, enabling also efficient propagation of diverse prion strains. This model allows studying how spontaneous prion diseases arise and provides powerful tools for investigating strain emergence, transmission barriers, and mechanisms ...
Hasier Eraña, Enric Vidal, Natalia Fernández‐Borges, Jorge M. Charco, Carlos M. Díaz‐Domínguez, Cristina Sampedro‐Torres‐Quevedo, Josu Galarza‐Ahumada, Eva Fernández‐Muñoz, Maitena San‐Juan‐Ansoleaga, Miguel Ángel Pérez‐Castro, Nuno Gonçalves‐Anjo, Patricia Piñeiro, Samanta Giler, Nora González‐Martín, Nuria L. Lorenzo, Africa Manero‐Azua, Guiomar Perez de Nanclares, Mariví Geijo, Manuel A. Sánchez‐Martín, Jesús R. Requena, Joaquín Castilla   +20 more
wiley   +1 more source

Soluble polymorphic bank vole prion proteins induced by co-expression of quiescin sulfhydryl oxidase in E. coli and their aggregation behaviors

Microbial Cell Factories, 2017
Background The infectious prion protein (PrPSc or prion) is derived from its cellular form (PrPC) through a conformational transition in animal and human prion diseases.
Romany Abskharon, Johnny Dang, Ameer Elfarash, Zerui Wang, Pingping Shen, Lewis S. Zou, Sedky Hassan, Fei Wang, Hisashi Fujioka, Jan Steyaert, Mentor Mulaj, Witold K. Surewicz, Joaquín Castilla, Alexandre Wohlkonig, Wen-Quan Zou   +14 more
doaj   +1 more source

Prion protein gene polymorphisms and accumulation of pathogenic prion protein (PrPSc) in a herd with previously confirmed scrapie cases

Bioscience Journal, 2015
Scrapie in sheep is associated with at least three polymorphisms in the prion protein gene (PRNP) on codons 136, 154, and 171. Countries where scrapie is endemic have been using breeding programs based on selection for the most resistant alleles. There are some PRNP genotyping data on sheep in Brazil, and scrapie has sporadically been observed since ...
Sotomaior, Cristina Santos, Driemeier, David, Leal, Juliano, Ollhoff, Rüdiger Daniel, Thomaz-Soccol, Vanete   +4 more
openaire   +5 more sources