Results 91 to 100 of about 97,942 (322)

Full atomistic model of prion structure and conversion

bioRxiv, 2018
Prions are unusual protein assemblies that propagate their conformationally-encoded information in absence of nucleic acids. The first prion identified, the scrapie isoform (PrPSc) of the cellular prion protein (PrPC), is the only one known to cause ...
G. Spagnolli, M. Rigoli, S. Orioli, Alejandro M. Sevillano, P. Faccioli, H. Wille, E. Biasini, J. Requena   +7 more
semanticscholar   +1 more source

Catalytic Amyloids: Turning Fibrils Into Biocatalysts

Chemistry – A European Journal, EarlyView.
Amyloids, traditionally associated with diseases, have emerged as versatile catalytic scaffolds. From natural amyloid sequences to bioinspired and de novo designs, we highlight strategies to construct catalytic active sites and anchor enzymes onto fibrils, creating versatile nanomaterials with tunable activities. ABSTRACT Amyloids have been regarded as
Alessandra Esposito, Linda Leone, Flavia Nastri, Angela Lombardi   +3 more
wiley   +1 more source

EGR1 Nuclear Condensates Promote Renal Cyst Development in Polycystic Kidney Disease

Exploration, EarlyView.
ABSTRACT Autosomal dominant polycystic kidney disease (ADPKD) is marked by aberrant cell proliferation driven by cAMP‐PKA and MAPK signaling pathways. EGR1, a transcription factor directly activated by the above two pathways, is critical in the over‐proliferation of tumor cells, which share similarities with cystic epithelial cells in ADPKD. This study
Chaoqun Ren, Zhaoxu Wu, Min Li, Yaqin Du, Hong Zhou, Yazhu Quan, Mengyao Xiong, Yiming Wang, Zhiwei Qiu, Shuai Zhu, Xiaowei Li, Jinzhao He, Cai Gao, Hui Cai, Tingting Li, Baoxue Yang   +15 more
wiley   +1 more source

Prion replication in organotypic brain slice cultures is distinct from in vivo inoculation and is species dependent

Acta Neuropathologica Communications
Cultured brain slices rapidly replicate murine prions, exhibit prion pathology, and are amenable towards drug discovery, but have not been infected with human prions.
Jessy A. Slota, Lise Lamoureux, Jennifer Myskiw, Kathy L. Frost, Sarah J. Medina, Dominic M. S. Kielich, Melanie Leonhardt, Gunjan Thapar, Ben A. Bailey-Elkin, Stephanie A. Booth   +9 more
doaj   +1 more source

Plasmonics‐Enhanced Characterization of Cervid PrP (87–114) Fragment Aggregates in Solution

Journal of Biophotonics, EarlyView.
Multimodal nanophotonics platform combining plasmon‐enhanced imaging, label‐free SERS, and optical staining reveals new insights into the aggregation of the cervid PrP (87–114) fragment, an amyloidogenic sequence linked to chronic wasting disease susceptibility.
Shinki Midha, Aishwarya Sriraman, Lyudmyla Dorosh, Holger Wille, Maria Stepanova   +4 more
wiley   +1 more source

The Endoplasmic Reticulum Chaperone GRP78/BiP Modulates Prion Propagation in vitro and in vivo

Scientific Reports, 2017
Prion diseases are fatal neurodegenerative disorders affecting several mammalian species, characterized by the accumulation of the misfolded form of the prion protein, which is followed by the induction of endoplasmic reticulum (ER) stress and the ...
Kyung-Won Park, Gyoung Eun Kim, R. Morales, Fabio Moda, I. Moreno-Gonzalez, Luis Concha-Marambio, Amy S. Lee, C. Hetz, C. Soto   +8 more
semanticscholar   +1 more source

A personal 360° view of applications of ‘biomimetic’ molecular recognition

Journal of Chemical Technology &Biotechnology, EarlyView.
Abstract Molecular recognition between biological molecules has formed the basis for innumerable applications in biotechnology for the last seven decades or so. Techniques such as affinity chromatography, solid‐phase and aqueous two‐phase extraction, affinity precipitation, biomimetic catalytic systems, biosensors and molecular imprinting all exploit ...
Christopher R Lowe
wiley   +1 more source

Genetic assessment of apolipoprotein E polymorphism and PRNP genotypes in rapidly progressive dementias in Pakistan

Prion
Rapidly progressive dementias (RPDs) are a type of fatal dementias that cause rapid progression of neuronal dysfunction. This study aimed to assess the prevalence of APOE genotypes (ε2, ε3, ε4) and PRNP mutations (E200K, M129V) in the general population ...
Urwah Rasheed, Minahil Khalid, Aneeqa Noor, Umar Saeed, Rizwan Uppal, Saima Zafar   +5 more
doaj   +1 more source

Characterization of mutations in PRNP (prion) gene and their possible roles in neurodegenerative diseases

Neuropsychiatric Disease and Treatment, 2018
Abnormal prion proteins are responsible for several fatal neurodegenerative diseases in humans and in animals, including Creutzfeldt–Jakob disease (CJD), Gerstmann–Sträussler–Scheinker disease, and fatal familial insomnia.
Eva Bagyinszky, V. Giau, Youngmin Youn, Seong Soo A An, Sangyun Kim   +4 more
semanticscholar   +1 more source

Prodromal Lewy Body Symptoms and α‐Synuclein Seeding in Idiopathic Olfactory Dysfunction

Movement Disorders Clinical Practice, EarlyView.
Abstract Background Early identification of pathological α‐synuclein deposition (αSynD) may improve understanding of Lewy body disorder (LBD) progression and enable timely disease‐modifying treatments. Objectives We investigated αSynD using a seed amplification assay and assessed prodromal LBD symptoms in individuals with idiopathic olfactory ...
Oskar Hoffmann McWilliam, Remarh Bsoul, Gunhild Waldemar, Steen Gregers Hasselbalch, Anja Hvid Simonsen, Marie Bruun, Laura Storm Næsborg Andersen, Christian von Buchwald, Kasper Aanæs, Christian K. Pedersen, Ida S.B. Andersen, Magne Bech, Clara Hageman Pinborg, Aušrinė Areškevičiūtė, Eva Løbner Lund, Kristian Steen Frederiksen   +15 more
wiley   +1 more source