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Showing 1 to 20 of 22 for “"Creutzfeldt-Jakob disease"”.
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Exploring Novel Immunodiagnostics for Prion Disease
Prion Diseases, or Transmissible Spongiform Encephalopathies (TSEs), are rapidly progressive and fatal neurodegenerative diseases of mammals. TSEs of global importance include Creutzfeldt-Jakob Disease (CJD) in humans, Chronic Wasting Disease (CWD) in cervids, and Bovine Spongiform Encephalopathy …
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Vergleichende Analyse der Gerstmann-Straeussler-Scheinker-Syndrom-assoziierten Mutation A117V mit der neuen pathogenen Mutation G114V des humanen Prion-Proteins in vivo und in vitro
… clinical symptoms overlap with GSS as well as Creutzfeldt-Jakob disease. Due to their close vicinity within the transmembrane domain of PrP and misincorporation of valine in both cases, PrP G114V and PrP A117V were investigated in comparative studies. Both mutations caused a similar cellular …
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Using Saccharomyces cerevisiae to characterise the in vivo effects of exposure to the prion-curing drug Tacrine and the fungal metabolite gliotoxin
… neurodegenerative disorders in mammals, such as Creutzfeldt-Jakob Disease (CJD) and Bovine Spongiform Encephalopathy (BSE). Fungal prions also exist, which provide a useful tool for studying the propagation of these non-mendelian genetic elements. Possibly the most widely-studied S. cerevisiae …
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Metabolic Profiling of Prions In The Gastro-Intestinal Tract
… <p><strong>Abstract</strong></p> <p>Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of debilitating neurodegenerative disorders that affect both humans and animals. They can be spread by horizontal transmission as seen in chronic wasting disease …
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ACUTE CELLULAR UPTAKE OF ABNORMAL PRION PROTEIN IS CELL TYPE AND SCRAPIE STRAIN INDEPENDENT
… (TSEs) are fatal neurodegenerative diseases that include Creutzfeldt-Jakob disease, bovine spongiform encephalopathy and sheep scrapie. TSE disease pathology and mechanisms within the central nervous system (CNS) of an infected host largely remains unclear. At the cellular level, the …
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Decontamination of prions, prion-associated amyloid and inefectivity from surgical stainless steel - implications for the risk of iatrogenic transmission of CJD
… nature of the infectious agent in prion diseases creates a<br/>significant challenge for decontamination services. It has been shown to be both resistant to<br/>standard methods of decontamination, used to inactivate viruses and bacteria, and to associate<br/>avidly with surgical …
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ACUTE CELLULAR UPTAKE OF ABNORMAL PRION PROTEIN IS CELL TYPE AND SCRAPIE STRAIN INDEPENDENT
… (TSEs) are fatal neurodegenerative diseases that include Creutzfeldt-Jakob disease, bovine spongiform encephalopathy and sheep scrapie. TSE disease pathology and mechanisms within the central nervous system (CNS) of an infected host largely remains unclear. At the cellular level, the …
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Preparation, characterisation and transcriptome analysis of RNA frm human vCJD brains.
The pathological mechanisms of variant Creutzfeldt-Jakob disease (vCJD) in the human brain remain poorly understood. Gene expression data may provide insight into the molecular mechanisms involved. This requires analysis of human postmortem brain tissue however; the variability in RNA preparations …
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Modelli murini transgenici di malattie da prioni per lo studio del ruolo fisiopatologico della proteina prionica.
Inherited prion diseases are linked to mutations in the prion protein (PrP) gene that are thought to favor the conformational conversion of PrP into a pathogenic misfolded isoform. Each mutation is associated with a distinct disease phenotype, which is profoundly affected by the Met/Val …
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Blood safety and resource allocation: economic analyses of donated blood safety initiatives
… travel due to possible exposure to variant Creutzfeldt-Jakob disease. Expanded European travel deferral will lead to the permanent deferral of 3,271 donors (95% Confidence Range, 2,600--3,973 donors) reducing the supply of blood by 3,141 units out of an approximate 94,000 unit annual supply …
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The role of germline and somatic nuclear and mitochondrial DNA variation in neurodegenerative disorders
… variants have all been hypothesised to increase disease risk or cause disease. Firstly, using a combination of exome sequencing and array genotyping on 1511 post-mortem brain samples within the MRC Brain Bank, we detected 61 monogenic cases of disease, 349 brains carrying disease risk factors, …
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STUDIO DEL RUOLO DELLA PROTEINA PRIONICA NELLA REGOLAZIONE DEL SONNO MEDIANTE L'UTILIZZO DI MODELLI MURINI TRANSGENICI
Introduction: an inherited form of Creutzfeldt-Jakob disease (CJD) is linked to the D178N/V129 mutation in the prion protein (PrP) gene. CJD is usually characterized by motor disorders, cognitive impairment and electroencephalographic alterations but recently sleep modification have been described. …
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Hybrid Particle-Nonwoven Membrane Materials for Bioseparations
… increased threat of blood-transfusion of variant Creutzfeldt-Jakob disease. This work characterizes the transport and binding properties of a novel hybrid particle-nonwoven membrane medium in which a polymeric chromatographic resin is entrapped between layers of a nonwoven polypropylene membrane …
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Identification of gene expression changes in Drosophila models of mammalian prion diseases
Prion diseases are fatal transmissible neurodegenerative diseases of humans and other animals. These include acquired prion diseases, such as scrapie in sheep, bovine spongiform encephalopathy in cattle and variant Creutzfeldt-Jakob disease in humans. Genetic prion diseases also occur in humans, …
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Induced-Pluripotent Stem-Derived Neuronal Progenitor Cells As A Novel Treatment For Neurodegenerative Diseases
… for patients affected with neurodegenerative diseases like Creutzfeldt-Jakob Disease (CJD). At this time there is no effective treatment or cure for CJD. The disease is inevitably fatal and affected people usually die within months of the appearance of the first clinical symptoms. Compelling …
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The subcellular trafficking of the prion protein: Characterisation of the function of the PrPc N-terminus
… encephalopathies (TSEs) are degenerative diseases of the central nervous system in humans and animals, and include Creutzfeldt-Jakob disease (CJD) in humans, scrapie in sheep and bovine spongiform encephalopathy in cattle. These spongiform encephalopathies can manifest as sporadic, …
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The Life of Prion: an investigation into the physiological role of a prion-like protein in the nematode Caenorhabditis elegans
For centuries, the threat of prion disease has plagued populations – whether it be in the form of scrapie ravaging through the sheep populations of Spain in the eighteenth century, fatal familial insomnia afflicting families in Italy, or an outbreak of Creutzfeldt-Jakob disease in the UK triggered …
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Characterization of the 37-kDa/67-kDa laminin receptor as the cell surface receptor for the cellular prion protein
… affect both, humans and animals. Human prion diseases occur in infectious, sporadic or genetic forms. The "protein only" hypothesis argues that the key event in the pathogenesis represents the conversion of the normal host protein, PrPc, into its pathogenic isoform PrPSc. Prion diseases have …
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Doxycyclin bei der sporadischen Creutzfeldt-Jakob-Krankheit
Die sporadische Creutzfeldt-Jakob-Krankheit (CJK) ist eine seltene neurodegenerative Erkrankung aus der Gruppe der Prionerkrankungen. Als zentraler pathophysiologischer Mechanismus wird die Ablagerung von pathologischem Prionprotein (PrPSc) angenommen, die zu Funktionsausfall und Zelltod im …
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