Human Prion Disease Surveillance in Washington State, 2006-2017
Sign Up to like & getrecommendations! 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
Prevalence of Surgical Procedures at Symptomatic Onset of Prion Disease
Sign Up to like & getrecommendations! 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
FDG‐PET patterns associate with survival in patients with prion disease
Sign Up to like & getrecommendations! Published in 2024 at "Annals of Clinical and Translational Neurology"
DOI: 10.1002/acn3.52230
Abstract: Prion disease classically presents with rapidly progressive dementia, leading to death within months of diagnosis. Advances in diagnostic testing have improved recognition of patients with atypical presentations and protracted disease courses, raising key questions surrounding… read more here.
Keywords: disease; fdg pet; prion disease; pet patterns ... See more keywords
A case report of genetic prion disease with two different PRNP variants
Sign Up to like & getrecommendations! 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
Volatile Anesthetic Sevoflurane Precursor 1,1,1,3,3,3-Hexafluoro-2-Propanol (HFIP) Exerts an Anti-Prion Activity in Prion-Infected Culture Cells
Sign Up to like & getrecommendations! 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
Electroretinogram (ERGs) and visual evoked potentials (VEPs) study in prion disease (CJD)
Sign Up to like & getrecommendations! 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
The most problematic symptoms of prion disease – an analysis of carer experiences
Sign Up to like & getrecommendations! 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
In vivo base editing extends lifespan of a humanized mouse model of prion disease
Sign Up to like & getrecommendations! Published in 2025 at "Nature Medicine"
DOI: 10.1038/s41591-024-03466-w
Abstract: Prion disease is a fatal neurodegenerative disease caused by the misfolding of prion protein (PrP) encoded by the PRNP gene. While there is currently no cure for the disease, depleting PrP in the brain is… read more here.
Keywords: disease; base editing; prion disease; prion ... See more keywords
Accelerated onset of CNS prion disease in mice co-infected with a gastrointestinal helminth pathogen during the preclinical phase
Sign Up to like & getrecommendations! 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
Identification of the novel polymorphisms and potential genetic features of the prion protein gene (PRNP) in horses, a prion disease-resistant animal
Sign Up to like & getrecommendations! 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
Structural effects of the highly protective V127 polymorphism on human prion protein
Sign Up to like & getrecommendations! 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