This article is a retrospective review about cases of diseases caused by prions cared for in our hospital over a 17 year period. The objective was to establish specific guidelines for treatment, norms and attitudes in order to achieve greater understanding, effectiveness and quality care for these patients as well as counsel all teams involved in their care, in light of the advances made in investigation over most recent years and the absence of publications in nursing journals about this topic.
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Vet Res
January 2025
UVSQ, INRAE, BREED, Université Paris-Saclay, 78350, Jouy-en-Josas, France.
Misfolding of the cellular PrP (PrP) protein causes prion disease, leading to neurodegenerative disorders in numerous mammalian species, including goats. A lack of PrP induces complete resistance to prion disease. The aim of this work was to engineer Alpine goats carrying knockout (KO) alleles of PRNP, the PrP-encoding gene, using CRISPR/Cas9-ribonucleoproteins and single-stranded donor oligonucleotides.
View Article and Find Full Text PDFNeurobiol Dis
January 2025
Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Toronto, Ontario, Canada; Department of Biochemistry, University of Toronto, Toronto, Ontario, Canada. Electronic address:
Bank voles are susceptible to prion strains from many different species, yet the molecular mechanisms underlying the ability of bank vole prion protein (BVPrP) to function as a universal prion acceptor remain unclear. Potential differences in molecular environments and protein interaction networks on the cell surface of brain cells may contribute to BVPrP's unusual behavior. To test this hypothesis, we generated knock-in mice that express physiological levels of BVPrP (M109 isoform) and employed mass spectrometry to compare the interactomes of mouse (Mo) PrP and BVPrP following mild in vivo crosslinking of brain tissue.
View Article and Find Full Text PDFVet Microbiol
January 2025
Department of Agricultural and Environmental Sciences, College of Agriculture, Tennessee State University, Nashville, TN, United States. Electronic address:
Porcine reproductive and respiratory syndrome (PRRS), caused by the highly variable PRRS virus (PRRSV), presents a significant challenge to the swine industry due to its pathogenic and economic burden. The virus evades host immune responses, particularly interferon (IFN) signaling, through various viral mechanisms. Traditional vaccines have shown variable efficacy in the field, prompting the exploration of novel vaccination strategies.
View Article and Find Full Text PDFJ Neurogenet
January 2025
Institute of Prion Diseases, MRC Prion Unit at University College London, London, UK.
Inherited prion diseases (IPD) secondary to mutations of the prion protein gene, exhibit diverse clinical phenotypes, capable of mimicking numerous primary neurodegenerative conditions. We describe the clinical phenotype and neuropathological findings in a family from County Limerick in Ireland presenting with Alzheimer's disease-like cognitive decline and motor symptoms caused by a novel missense mutation of This mutation occurs in the central lysine cluster (CLC; codon 101-110), resulting in substitution of threonine with isoleucine at codon 107 (T107I). This case series highlights that IPD can be hard to distinguish from overlapping clinical syndromes seen in other neurodegenerative diseases.
View Article and Find Full Text PDFJ Neurol Sci
January 2025
Laboratory of Molecular Biology and Genetics, Postgraduate Program of Health Sciences, São Francisco University, Bragança Paulista, São Paulo, Brazil; Laboratory of Clinical and Molecular Microbiology, Postgraduate Program of Health Sciences, São Francisco University, Bragança Paulista, São Paulo, Brazil; LunGuardian Research Group - Epidemiology of Respiratory and Infectious Diseases, Postgraduate Program of Health Sciences, São Francisco University, Bragança Paulista, São Paulo, Brazil. Electronic address:
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