Global ETD Search
Search theses and dissertations gathered from participating repositories worldwide. Every result links back to the library that holds it. No account is needed.
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Showing 1 to 18 of 18 for “"CJD"”.
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STUDIO DEL RUOLO DELLA PROTEINA PRIONICA NELLA REGOLAZIONE DEL SONNO MEDIANTE L'UTILIZZO DI MODELLI MURINI TRANSGENICI
… 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. Moreover a …
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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
… sporadic forms of Creutzfeldt-Jakob Disease (CJD) suggests deposition of the infectious<br/>agent across a wide range of extraneural, lymphoid tissues, as well as in the skeletal muscle<br/>and blood. Coupled with the potential for asymptomatic carriers, there is a significant risk …
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Modelli murini transgenici di malattie da prioni per lo studio del ruolo fisiopatologico della proteina prionica.
… insomnia (FFI) and Creutzfeldt-Jakob disease (CJD178), two clinically and neuropathologically distinct diseases linked to the D178N mutation in the gene encoding PrP; D178N/M129 segregates with FFI, while D178N/V129 is associated with CJD. We have engineered transgenic (Tg) mice to express …
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Übertragung von BSE auf nicht humane Primaten als Modell für die variante Creutzfeldt-Jakob Erkrankung (vCJD) im Menschen
… an der varianten Creutzfeldt-Jakob Erkrankung (vCJD) zu erkranken. Als Tiermodell für den Menschen dienen Javaneraffen (Macaca fascicularis). In der Studie wurden Gruppen von je 6 nicht humanen Primaten intracerebral oder oral mit Verdünnungsreihen aus Hirnhomogenat BSE-infizierter Rinder …
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Induced-Pluripotent Stem-Derived Neuronal Progenitor Cells As A Novel Treatment For Neurodegenerative Diseases
… 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 evidence indicate that the hallmark event …
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Exploring Novel Immunodiagnostics for Prion Disease
… importance include Creutzfeldt-Jakob Disease (CJD) in humans, Chronic Wasting Disease (CWD) in cervids, and Bovine Spongiform Encephalopathy (BSE) in cattle. These diseases occur when the normal cellular prion protein (PrPC) misfolds, producing the infectious isoform PrPSc,which can readily …
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Assessing the role of Hsp70 in prion propagation in Saccharomyces cerevisiae
… including; BSE, vBSE, scrapie and CJD. Prions are also present in fungi. There have been a number of prion proteins discovered in the yeast Saccharomyces cerevisiae. Probably the most studied of these is the [PSI+], which is the prion form of the protein Sup35, which is required for …
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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
… 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 prion is [PSI+], which is the …
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Design and synthesis of constrained dipeptide units for use as β-sheet promoters
… disease (AD), Creutzfeldt-Jacob disease (CJD) and bovine spongiform encephalopathy. Understanding beta-sheet formation and the factors that stabilize beta-sheet structure may serve as a basis for future drug design. The extended structure of a beta-sheet can be stabilized by constrained …
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The role of germline and somatic nuclear and mitochondrial DNA variation in neurodegenerative disorders
… LAMA5 associated with Creutzfeldt-Jakob disease (CJD), and fourthly, we determine that at least 1% of the population carry high level somatic protein-coding mutations affecting at least 10% of cells within the brain. Subsequently, additional focussed deep-sequencing studies revealed that several …
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Deciphering Functional Significance of Substrate-Binding Domain of Ssa1 on Heat-Shock Response and Prion Propagation
… and the prion disease Creutzfeldt-Jacob Disease (CJD) are also intimately linked to Hsp70. Structurally, Hsp70 is comprised of two domains: nucleotide-binding domain (NBD) and substrate-binding domain (SBD). In this work, a well-established yeast system and a combination of computational biology, …
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Identification of gene expression changes in Drosophila models of mammalian prion diseases
… in the prion protein (PrP) gene, such as genetic CJD (gCJD) and fatal familial insomnia. Prion diseases are caused by the misfolding of the cellular isoform of PrP (PrPC) into the disease-associated isoform (PrPSc). Prion inoculated wild-type and PrP transgenic mice have been used to model …
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The subcellular trafficking of the prion protein: Characterisation of the function of the PrPc N-terminus
… 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, familial and acquired disorders and are caused by the conformational alteration of the non-pathogenic …
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Characterization of the 37-kDa/67-kDa laminin receptor as the cell surface receptor for the cellular prion protein
… insomnia (FFI) and Creutzfeldt-Jakob disease (CJD) reveal proteinase K (PK) resistance, one of the most typical biochemical properties characteristic for the infectious scrapie isoform of the prion protein. The subcellular location of both PrP mutants at the cell surface and in intracellular …
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Intravital diagnostics of neurodegenerative diseases
Prionopatie, taktiež nazývané transmisívne spongiformné encefalopatie (TSE), a synukleínopatie predstavujú skupinu neurodegeneratívnych ochorení, ktoré sú asociované s akumuláciou nesprávne zložených proteínov (prión a α-synukleín) prevažne v centrálnom nervovom systéme. Kvôli absencii spoľahlivého …
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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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Risikofaktoren der sporadischen Creutzfeldt-Jakob-Krankheit
Die sporadische Creutzfeldt-Jakob-Krankheit (sCJK) macht den größten Anteil an den humanen Transmissiblen Spongiformen Enzephalopathien aus, ihre Ursache gibt noch immer viele Rätsel auf. In der vorliegenden Arbeit wurde eine Fall-Kontroll-Studie mit 1155 deutschen sicheren und wahrscheinlichen …
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Das zelluläre Prionprotein im Liquor cerebrospinalis von Patienten mit verschiedenen neurologischen Erkrankungen
In der vorliegenden Arbeit wurden 317 Liquorproben von Patienten mit verschiedenen neurodegenerativen Erkrankungen wie CJK, Alzheimer-Demenz, Demenz mit Lewykörperchen, Morbus Parkinson und Normaldruckhydrozephalus sowie von Patienten mit nicht-neurodegenerativen Erkrankungen wie Multipler …