Results 41 to 50 of about 75,780 (211)

Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding [PDF]

open access: yes, 2010
Transmissible spongiform encephalopathies, or prion diseases, are caused by misfolding and aggregation of the prion protein PrP. These diseases can be hereditary in humans and four of the many disease-associated missense mutants of PrP are in the ...
Valerie Daggett   +3 more
core   +1 more source

The Molecular Pathology of Prion Diseases [PDF]

open access: yes, 2004
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal ...
Vassallo, Neville   +2 more
core  

Prion protein in the cerebrospinal fluid of healthy and naturally scrapie-affected sheep [PDF]

open access: yes, 2006
The aim of this study was to characterize the cerebrospinal fluid (CSF) prion protein (PrP) of healthy and naturally scrapie-affected sheep. The soluble form of CSF PrPC immunoblotted with an anti-octarepeat and an anti-C terminus mAb showed two isoforms
Vé Ronique Gayrard   +13 more
core   +1 more source

Canine detection of chronic wasting disease (CWD) in laboratory and field settings

open access: yesPrion, 2023
Chronic wasting disease (CWD) is a fatal transmissible spongiform encephalopathy that affects both free-ranging and farmed cervid species, including mule deer, white-tailed deer, and elk (Odocoileus hemionus, Odocoileus virginianus, and Cervus canadensis)
Amritha Mallikarjun   +11 more
doaj   +1 more source

A novel protective prion protein variant that colocalizes with kuru exposure. [PDF]

open access: yes, 2009
BACKGROUND: Kuru is a devastating epidemic prion disease that affected a highly restricted geographic area of the Papua New Guinea highlands; at its peak, it predominantly affected adult women and children of both sexes.
Whittaker, John   +26 more
core   +1 more source

­Classifying prion and prion-like phenomena [PDF]

open access: yesPrion, 2014
The universe of prion and prion-like phenomena has expanded significantly in the past several years. Here, we overview the challenges in classifying this data informatically, given that terms such as "prion-like", "prion-related" or "prion-forming" do not have a stable meaning in the scientific literature.
Harbi, Djamel, Harrison, Paul M
openaire   +2 more sources

Molecular diagnostics helps to identify distinct subgroups of spinal astrocytomas

open access: yesActa Neuropathologica Communications, 2021
Primary spinal cord astrocytomas are rare, hence few data exist about the prognostic significance of molecular markers. Here we analyze a panel of molecular alterations in association with the clinical course.
Annamaria Biczok   +9 more
doaj   +1 more source

Serial changes in regional cerebral blood flow in Gerstmann–Sträussler–Scheinker disease caused by a Pro-to-Leu mutation at codon 105 in the prion protein gene

open access: yesPrion, 2023
Gerstmann–Sträussler–Scheinker disease with a Pro-to-Leu substitution at codon 105 in the prion protein gene (GSS-P105L) is a rare variant of human genetic prion disease.
Honami Kawai   +3 more
doaj   +1 more source

Primary glia cells from bank vole propagate multiple rodent-adapted scrapie prions

open access: yesScientific Reports, 2022
Since the beginning prion research has been largely dependent on animal models for deciphering the disease, drug development or prion detection and quantification. Thereby, ethical as well as cost and labour-saving aspects call for alternatives in vitro.
Karla A. Schwenke   +4 more
doaj   +1 more source

Expression of the cellular prion protein by mast cells in the human carotid body

open access: yesPrion, 2023
Prion diseases are fatal neurologic disorders that can be transmitted by blood transfusion. The route for neuroinvasion following exposure to infected blood is not known.
Gregory D. Sweetland   +4 more
doaj   +1 more source

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