Articles with "prion disease" as a keyword



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Human Prion Disease Surveillance in Washington State, 2006-2017

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Published in 2020 at "JAMA Network Open"

DOI: 10.1001/jamanetworkopen.2020.20690

Abstract: Key Points Question What are the results of human prion disease surveillance in Washington state? Findings In this cross-sectional study using state surveillance data from 2006 to 2017, 143 human prion disease cases were detected,… read more here.

Keywords: state; surveillance; prion; disease ... See more keywords
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Prevalence of Surgical Procedures at Symptomatic Onset of Prion Disease

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Published in 2022 at "JAMA Network Open"

DOI: 10.1001/jamanetworkopen.2022.1556

Abstract: This case-control study examines the frequency of invasive procedures at the onset of prion disease symptoms to determine the scope of the risk of contamination to future patients. read more here.

Keywords: prevalence surgical; prion disease; onset prion; surgical procedures ... See more keywords
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A case report of genetic prion disease with two different PRNP variants

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Published in 2020 at "Molecular Genetics & Genomic Medicine"

DOI: 10.1002/mgg3.1134

Abstract: Prion diseases are a group of lethal neurodegenerative conditions that occur when the normal, cellular form of the prion protein (PrPC) is converted into an abnormal, scrapie, form of the protein (PrPSc). Disease may be… read more here.

Keywords: prnp variants; prion; disease; case ... See more keywords
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Volatile Anesthetic Sevoflurane Precursor 1,1,1,3,3,3-Hexafluoro-2-Propanol (HFIP) Exerts an Anti-Prion Activity in Prion-Infected Culture Cells

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Published in 2021 at "Neurochemical Research"

DOI: 10.1007/s11064-021-03344-8

Abstract: Prion disease is a neurodegenerative disorder with progressive neurologic symptoms and accelerated cognitive decline. The causative protein of prion disease is the prion protein (PrP), and structural transition of PrP from the normal helix rich… read more here.

Keywords: hfip; prion; prion disease; anesthetic sevoflurane ... See more keywords
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Electroretinogram (ERGs) and visual evoked potentials (VEPs) study in prion disease (CJD)

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Published in 2019 at "Clinical Neurophysiology"

DOI: 10.1016/j.clinph.2018.09.079

Abstract: Literature reported on ERGs and VEPs changes in patients with prion disease (CJD). To characterize the extent of ERG and VEPs involvement in confirmed CJD; to assess ERGs and VEPs diagnostic usefulness in the workout… read more here.

Keywords: prion disease; disease cjd; ergs veps; cjd ... See more keywords
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The most problematic symptoms of prion disease – an analysis of carer experiences

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Published in 2019 at "International Psychogeriatrics"

DOI: 10.1017/s1041610218001588

Abstract: ABSTRACT Objectives: Prion diseases are rare dementias that most commonly occur sporadically, but can be inherited or acquired, and for which there is no cure. We sought to understand which prion disease symptoms are most… read more here.

Keywords: prion; prion disease; people prion; problematic symptoms ... See more keywords
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Accelerated onset of CNS prion disease in mice co-infected with a gastrointestinal helminth pathogen during the preclinical phase

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Published in 2020 at "Scientific Reports"

DOI: 10.1038/s41598-020-61483-4

Abstract: Prion infections in the central nervous system (CNS) can cause extensive neurodegeneration. Systemic inflammation can affect the progression of some neurodegenerative disorders. Therefore, we used the gastrointestinal helminth pathogen Trichuris muris to test the hypothesis… read more here.

Keywords: cns prion; prion; cns; prion disease ... See more keywords
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Identification of the novel polymorphisms and potential genetic features of the prion protein gene (PRNP) in horses, a prion disease-resistant animal

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Published in 2020 at "Scientific Reports"

DOI: 10.1038/s41598-020-65731-5

Abstract: Prion diseases, a protein misfolded disorder (PMD) caused by misfolded prion protein (PrPSc), present in a wide variety of hosts, ranging from ungulates to humans. To date, prion infections have not been reported in horses,… read more here.

Keywords: horse; prion; prion disease; prion protein ... See more keywords
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Structural effects of the highly protective V127 polymorphism on human prion protein

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Published in 2020 at "Communications Biology"

DOI: 10.1038/s42003-020-01126-6

Abstract: Prion diseases, a group of incurable, lethal neurodegenerative disorders of mammals including humans, are caused by prions, assemblies of misfolded host prion protein (PrP). A single point mutation (G127V) in human PrP prevents prion disease,… read more here.

Keywords: prion; human prion; prion disease; prion protein ... See more keywords
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Diagnostic accuracy of cerebrospinal fluid biomarkers in genetic prion diseases

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Published in 2022 at "Brain"

DOI: 10.1093/brain/awab350

Abstract: Abstract Genetic prion diseases are a rare and diverse group of fatal neurodegenerative disorders caused by pathogenic sequence variations in the prion protein gene, PRNP. Data on CSF biomarkers in patients with genetic prion diseases… read more here.

Keywords: prion; genetic prion; prion disease; prion diseases ... See more keywords
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The Braak hypothesis in prion disease with a focus on Creutzfeldt–Jakob disease

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Published in 2020 at "Neuropathology"

DOI: 10.1111/neup.12654

Abstract: This review considers whether the Braak hypothesis on protein propagation could account for prion disease, particularly Creutzfeldt–Jakob disease (CJD). In CJD, we can speculate on the pathological onset region to some degree in reference to… read more here.

Keywords: prion disease; deposition; braak hypothesis; disease ... See more keywords