Results 101 to 110 of about 45,606 (277)

Characterization of variably protease-sensitive prionopathy by capillary electrophoresis

open access: yesScientific Reports
Variably Protease Sensitive Prionopathy (VPSPr) is a rare human prion disease that, like Creutzfeldt-Jakob disease (CJD), results in the deposition of abnormally folded prion protein aggregates in the brain and is ultimately fatal.
Jennifer Myskiw   +7 more
doaj   +1 more source

Transmission and Characterization of Creutzfeldt–Jakob Disease and Chronic Wasting Disease in the North American Deer Mouse

open access: yesViruses
Prion transmission into rodents is essential for understanding prion strains. However, it is often limited by a “species barrier” that makes transmission challenging and complicates the study of animal and human prion diseases. Here, we report that North
Jennifer Myskiw   +7 more
doaj   +1 more source

An overview of animal prion diseases

open access: yesVirology Journal, 2011
Prion diseases are transmissible neurodegenerative conditions affecting human and a wide range of animal species. The pathogenesis of prion diseases is associated with the accumulation of aggregates of misfolded conformers of host-encoded cellular prion ...
Imran Muhammad, Mahmood Saqib
doaj   +1 more source

Development of a sensitive cell culture system to assess prion infectivity and the efficacy of prion decontamination technologies

open access: yes, 2012
Creutzfeldt-Jakob disease (CJD) can be iatrogenically transmitted during transplants, grafts and transfusions from CJD infected donors and also contaminated surgical instruments.
Secker, Thomas
core  

The crystal structure of the globular domain of sheep prion protein

open access: yes, 2004
The prion protein PrP is a naturally occurring polypeptide that becomes transformed from a normal conformation to that of an aggregated form, characteristic of pathological states in fatal transmissible spongiform conditions such as Creutzfeld–Jacob ...
Vasisht N   +28 more
core   +1 more source

Prion diseases are efficiently transmitted by blood transfusion in sheep [PDF]

open access: yes, 2008
The emergence of variant Creutzfeld-Jakob disease, following on from the bovine spongiform encephalopathy (BSE) epidemic, led to concerns about the potential risk of iatrogenic transmission of disease by blood transfusion and the introduction of costly ...
Foster, J.   +8 more
core   +1 more source

Imaging patterns and genetic associations of brain atrophy across distinct symptom stages in Parkinson's disease

open access: yesNeuroprotection, EarlyView.
Early disease stages showed limited cortical atrophy and enrichment of synaptic and calcium signaling pathways, whereas advanced stages demonstrated widespread cortical degeneration associated with immune activation and extracellular matrix remodeling.
Yi Ji   +6 more
wiley   +1 more source

The end of the BSE saga: do we still need surveillance for human prion diseases?

open access: yesSwiss Medical Weekly, 2015
The epidemics of classical bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) related to BSE-infected food are coming to an end.
Herbert Budka, Robert G. Will
doaj   +1 more source

RNA‐binding protein RBMS1: A new target for cancer diagnosis and treatment

open access: yesPrecision Medical Sciences, EarlyView.
This schematic illustrates the diverse regulatory roles of RNA‐binding motif single‐stranded interacting protein 1 (RBMS1) across multiple human cancers. In breast cancer, RBMS1 stabilizes B4GALT1 mRNA to promote PD‐L1 glycosylation, modulating tumor immune escape and immunotherapy efficacy.
Xingda Run   +7 more
wiley   +1 more source

Epilepsia partialis continua as the presenting manifestation of Creutzfeldt–Jakob disease: A video‐polygraphic clinical vignette

open access: yes
Epileptic Disorders, EarlyView.
Roberta Cutellè   +8 more
wiley   +1 more source

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