Results 21 to 30 of about 48,246 (290)

Prions: Portable prion domains [PDF]

Current Biology, 2000
Self-propagating abnormal proteins, prions, have been identified in yeast; asparagine/glutamine-rich 'prion domains' within these proteins can inactivate the linked functional domains; new prion domains and reporters have been used to make 'synthetic prions', leading to discoveries of new natural prions.
Wickner, R.B., Taylor, K.L., Edskes, H.K., Maddelein, M-L.   +3 more
openaire   +2 more sources

The Standard Scrapie Cell Assay: Development, Utility and Prospects

Viruses, 2015
Prion diseases are a family of fatal neurodegenerative diseases that involve the misfolding of a host protein, PrPC. Measuring prion infectivity is necessary for determining efficacy of a treatment or infectivity of a prion purification procedure; animal
Jacques van der Merwe, Judd Aiken, David Westaway, Debbie McKenzie   +3 more
doaj   +1 more source

Medium-throughput zebrafish optogenetic platform identifies deficits in subsequent neural activity following brief early exposure to cannabidiol and Δ9-tetrahydrocannabinol

Scientific Reports, 2021
In light of legislative changes and the widespread use of cannabis as a recreational and medicinal drug, delayed effects of cannabis upon brief exposure during embryonic development are of high interest as early pregnancies often go undetected.
Richard Kanyo, Md Ruhul Amin, Laszlo F. Locskai, Danika D. Bouvier, Alexandria M. Olthuis, W. Ted Allison, Declan W. Ali   +6 more
doaj   +1 more source

Transmission of Prions [PDF]

The Journal of Infectious Diseases, 2002
The “protein only” hypothesis states that the infectious agent causing transmissible spongiform encephalopathies is a conformational isomer of PrP, a host protein predominantly expressed in brain, and is strongly supported by many lines of evidence. Prion diseases are so far unique among conformational diseases in that they are transmissible, not only ...
C, Weissmann, M, Enari, P-C, Klöhn, D, Rossi, E, Flechsig   +4 more
openaire   +4 more sources

The CNS in inbred transgenic models of 4-repeat Tauopathy develops consistent tau seeding capacity yet focal and diverse patterns of protein deposition

Molecular Neurodegeneration, 2017
Background MAPT mutations cause neurodegenerative diseases such as frontotemporal dementia but, strikingly, patients with the same mutation may have different clinical phenotypes.
Ghazaleh Eskandari-Sedighi, Nathalie Daude, Hristina Gapeshina, David W. Sanders, Razieh Kamali-Jamil, Jing Yang, Beipei Shi, Holger Wille, Bernardino Ghetti, Marc I. Diamond, Christopher Janus, David Westaway   +11 more
doaj   +1 more source

Early preclinical detection of prions in the skin of prion-infected animals

Nature Communications, 2019
A definitive pre-mortem diagnosis of prion disease depends on brain biopsy for prion detection currently and no validated alternative preclinical diagnostic tests have been reported to date.
Zerui Wang, M. Manca, Aaron Foutz, M. Camacho, G. J. Raymond, B. Race, C. Orrú, Jue Yuan, Pingping Shen, Baiya Li, Yue Lang, Johnny Dang, Alise Adornato, K. Williams, Nicholas R. Maurer, P. Gambetti, Bin Xu, W. Surewicz, R. Petersen, Xiao-Ping Dong, B. Appleby, B. Caughey, Li Cui, Qingzhong Kong, W. Zou   +24 more
semanticscholar   +1 more source

High diagnostic value of second generation CSF RT-QuIC across the wide spectrum of CJD prions

Scientific Reports, 2017
An early and accurate in vivo diagnosis of rapidly progressive dementia remains challenging, despite its critical importance for the outcome of treatable forms, and the formulation of prognosis.
A. Franceschini, Simone Baiardi, A. Hughson, Neil McKenzie, F. Moda, Marcello Rossi, S. Capellari, A. Green, G. Giaccone, B. Caughey, Piero Parchi   +10 more
semanticscholar   +1 more source

Autophagy regulates exosomal release of prions in neuronal cells

Journal of Biological Chemistry, 2018
Prions are protein-based infectious agents that autocatalytically convert the cellular prion protein PrPC to its pathological isoform PrPSc.
Basant A. Abdulrahman, D. Abdelaziz, H. Schatzl   +2 more
semanticscholar   +1 more source

Octarepeat region flexibility impacts prion function, endoproteolysis and disease manifestation

EMBO Molecular Medicine, 2015
The cellular prion protein (PrPC) comprises a natively unstructured N‐terminal domain, including a metal‐binding octarepeat region (OR) and a linker, followed by a C‐terminal domain that misfolds to form PrPSc in Creutzfeldt‐Jakob disease.
Agnes Lau, Alex McDonald, Nathalie Daude, Charles E Mays, Eric D Walter, Robin Aglietti, Robert CC Mercer, Serene Wohlgemuth, Jacques van der Merwe, Jing Yang, Hristina Gapeshina, Chae Kim, Jennifer Grams, Beipei Shi, Holger Wille, Aru Balachandran, Gerold Schmitt‐Ulms, Jiri G Safar, Glenn L Millhauser, David Westaway   +19 more
doaj   +1 more source

Deposition pattern and subcellular distribution of disease-associated prion protein in cerebellar organotypic slice cultures infected with scrapie

Frontiers in Neuroscience, 2015
Organotypic cerebellar slices represent a suitable model for characterizing and manipulating prion replication in complex cell environments. Organotypic slices recapitulate prion pathology and are amenable to drug testing in the absence of a blood-brain ...
Hanna eWolf, Andre eHossinger, Andrea eFehlinger, Sven eBuettner, Valerie eSim, Debbie eMcKenzie, Ina Maja Vorberg, Ina Maja Vorberg   +7 more
doaj   +1 more source