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Dissertations / Theses on the topic 'Creutzfeldt-Jakob disease'

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Published: 4 June 2021

Last updated: 26 July 2025

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1

Heslin, Donna E. "Lesion profiling in Creutzfeldt-Jakob disease." Thesis, National Library of Canada = Bibliothèque nationale du Canada, 2001. http://www.collectionscanada.ca/obj/s4/f2/dsk3/ftp04/MQ58829.pdf.

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2

Bunn, Tristan. "Prion protein biochemistry in Creutzfeldt-Jakob disease." Thesis, University of Edinburgh, 2003. http://hdl.handle.net/1842/23281.

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3

Macleod, Margaret-Ann. "The clinico-pathological phenotype of sporadic Creutzfeldt-Jakob disease." Thesis, University of Edinburgh, 2002. http://hdl.handle.net/1842/28507.

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Creutzfeldt-Jakob Disease (CJD) is a rare neurodegenerative disorder of the human central nervous system. It occurs in four main forms, defined essentially according to aetiology: sporadic, variant, iatrogenic and familial. All forms of the disease are characterised by the deposition of an abnormal cellular protein, the prion protein (PrP^sc), within the brain. Definitive diagnosis depends on identifying this along with other neuropathological changes such as spongiform degeneration and astrocytic gliosis. To date variant CJD has followed a relatively stereotyped clinical course with fairly co

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4

Cordery, R. J. "The early diagnosis and management of Creutzfeldt-Jakob disease." Thesis, University College London (University of London), 2004. http://discovery.ucl.ac.uk/1446588/.

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This thesis describes work undertaken to improve the early diagnosis of variant Creutzfeldt-Jakob disease (vCJD), using existing clinical and research tools. Twenty-one cases referred to the National Hospital for Neurology and Neurosurgery and St. Mary's Hospital, London with suspected vCJD completed participation in the study. Fifteen cases were confirmed with definite or probable vCJD and six were given alternative diagnoses. These six cases with alternative diagnoses formed a control group. Further controls were recruited from patients referred with sporadic and familial forms of prion dise

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5

Mackay, Graham. "Clinico-pathological phenotypes of sporadic CJD in relation to PpPres type." Thesis, University of Aberdeen, 2012. http://digitool.abdn.ac.uk:80/webclient/DeliveryManager?pid=202124.

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6

Zeidler, Martin. "A new variant of Creutzfeldt-Jakob disease in the United Kingdom." Thesis, University of Southampton, 2002. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.274425.

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7

de, Vries Kay. "Caring for the person with variant Creutzfeldt-Jakob disease within the hospice service." Thesis, University of Surrey, 2006. http://epubs.surrey.ac.uk/842755/.

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This thesis explores the experience of caring for people with the new diseases of variant Creutzfeldt-Jakob disease (CJD) by hospice services. The experiences of the hospice staff and carers of five people, who had died, or were dying, as a result of variant CJD, at three different hospice sites in the South of England were explored. Data from four focus groups and 11 interviews, with hospice staff (included health care assistants, nurses, doctors and social workers) and family members, plus hospice patient records were analysed. Media material was also examined and used to inform the study. T

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8

Choi, Young Pyo. "PrPSc complexity in different forms of Creutzfeldt-Jakob disease identified using biochemical approaches." Thesis, University of Edinburgh, 2010. http://hdl.handle.net/1842/4798.

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Transmissible spongiform encephalopathies (TSEs) or prion diseases are a group of fatal neurodegenerative diseases affecting humans and animal species. Prion diseases are characterized by the conversion of the host encoded prion protein (PrPC) into a disease-associated isoform (PrPSc), which (according to the prion hypothesis) is thought to be the main component of the infectious agent. PrPSc has been traditionally distinguished from PrPC by its biochemical properties, such as partial resistance to proteolysis and detergent-insolubility. In the absence of a foreign nucleic acid genome associat

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9

Betmouni, Samar. "Inflammatory response in a mouse model of scrapie." Thesis, University of Oxford, 1997. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.361753.

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10

Ironside, James Wilson. "Neuropathology and molecular biology of iatrogenic Creutzfeldt-Jakob disease in UK human growth hormone recipients." Thesis, University of Edinburgh, 2017. http://hdl.handle.net/1842/28933.

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Creutzfeldt-Jakob disease (CJD) is the commonest form of human prion disease and occurs in sporadic, genetic and acquired forms. The causative agents (prions) appear to be composed entirely of a modified host protein, the prion protein, which undergoes misfolding to a disease-associated isoform closely associated with infectivity that is resistant to conventional methods of decontamination. Prions can be transmitted from one individual to another by medical and surgical procedures, resulting in iatrogenic CJD (iCJD). The commonest cause of iCJD is the inoculation of cadaveric pituitary-derived

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11

Douet, Jean-Yves. "Perméabilité des barrières de transmission et évaluation du risque iatrogène associé aux agents responsables des Encéphalopathies Spongiformes Transmissibles." Thesis, Toulouse, INPT, 2015. http://www.theses.fr/2015INPT0033/document.

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Les Encéphalopathies Spongiformes transmissibles (EST) sont des maladies neurodégénératives fatales caractérisées par l’accumulation d’un conformère anormal (PrPSc) d’une protéine de l’hôte (PrP). Chez l’homme, plusieurs centaines de cas de transmissions iatrogènes de la maladie de Creutzfeldt Jakob (MCJ) ont été identifiées, notamment chez des patients ayant fait l’objetd’ une greffe de dure-mère, de cornée ou des injections d’hormones de croissance extractives. Plus récemment, plusieurs cas du variant de la maladie de Creutzfeldt Jakob (vMCJ) ont été observés chez des patients transfusés ave

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12

De, Silva Rajith Nilantha. "A correlative study of the clinical, pathological and molecular biological features of Creutzfeldt-Jakob disease." Thesis, University of Edinburgh, 1998. http://hdl.handle.net/1842/27887.

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A systematic study of all patients with pathologically or electrophysiologically confirmed Creutzfeld-Jakob disease (CJD) referred to the U.K. national surveillance unit between May, 1990 and April, 1994 has been undertaken. The numbers of sporadic, familial and iatrogenic cases were 144, 14 and 12 respectively. Sporadic CJD cases had a median age at presentation of 65 years, and a median disease duration of 4 months. Familial cases (associated with mutations of the open reading frame of the prion protein gene, PRNP ORF) presented 10 years earlier and had disease durations which were twice as

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13

Cooper, J. D. "Statistical modelling of UK dietary exposure to bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease." Thesis, University of Cambridge, 2003. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.597975.

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Surprisingly, few attempts have been made to ascertain the data required to quantify human dietary exposure to BSE. After investigations, the primary sources of exposure were assumed to have been through the consumption of burgers, sausages and other meat products containing beef mechanically recovered meat (MRM) and head meat, which were potentially contaminated respectively with BSE infected spinal cord and dorsal root ganglia, and brain. Risk analyses for contamination are conducted and meat product destination and calendar year production are estimated using information collected from repo

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14

Nakatani, Eiji. "Specific clinical signs and symptoms are predictive of clinical course in sporadic Creutzfeldt-Jakob disease." Kyoto University, 2016. http://hdl.handle.net/2433/217720.

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15

Cooper, Sarah. "The clinical features and diagnosis of sporadic Creutzfeldt-Jakob disease in the United Kingdom, 1990-2002." Thesis, University of Edinburgh, 2005. http://hdl.handle.net/1842/26411.

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Background: Sporadic Creutzfeldt-Jakob disease (sCJD) is an invariably fatal spongiform encephalopathy that has a worldwide incidence of approximately 1/1,000000 per year. Prospective surveillance has been in place in the UK since 1990, coordinated by the National CJD Surveillance Unit (NCJDSU). Accurate surveillance of CJD is important not only in detecting changes in patterns of disease and predicting trends but also in instituting protective public health measures. Aims of the study: To define circumstances where making a clinical diagnosis of sCJD is potentially problematic. To explore way

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16

Correia, Susana Margarida da Silva. "Altered regulatory response of Rab7a and Rab9 in MM1 and VV2 subtype of Creutzfeldt-Jakob disease." Master's thesis, Universidade de Aveiro, 2015. http://hdl.handle.net/10773/15769.

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Mestrado em Biologia Molecular e Celular<br>The present study was undertaken to identify proteins interacting with PrPC that could provide new insights into its physiological functions and pathological role. We performed a target search for lysosomal network protein, Rab7a and Rab9, in frontal cortex and cerebellum of human brain from patients with sCJD-MM1 and sCJD-VV2. The intracellular level of Rab7a was increased significantly, when compared with healthy age-matched control. Interactions of PrPC and Rab7a/Rab9 were further investigated by using confocal laser scanning microscopy. Immunoflu

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17

Chohan, Gurjit Kaur. "A correlative study of the clinical, pathological and biochemical features of sporadic Creutzfeldt-Jakob Disease, 2000-2006." Thesis, University of Leicester, 2013. http://hdl.handle.net/2381/28449.

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Historically various CJD syndromes have been described with a spectrum of clinical presentations. Sporadic CJD (sCJD) is the most common form of human transmissible spongiform encephalopathy (TSE) and is characterised by accumulation of pathological prion protein (PrP) in the central nervous tissue. Classification criteria have changed with the advent of novel diagnostic tests over the last decade. The subclassification according to the codon 129 genotype and prion protein (PrP) type in the brain has led to the identification of six sCJD subtypes, but whether different phenotypes are related t

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18

Peckeu, Laurène. "Physiopathologie des formes infectieuses de maladies à prions humaines : étude des formes iatrogènes secondaires à un traitement par l'hormone de croissance." Thesis, Paris 6, 2017. http://www.theses.fr/2017PA066583/document.

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Les maladies à prions sont des maladies neurodégénératives et transmissibles. Elles sont à l'origine de formes infectieuses comme la maladie de Creutzfeldt-Jakob iatrogène secondaire à un traitement par hormone de croissance d'origine humaine (MCJ post-hGH). La compréhension des facteurs gouvernant la physiopathologie de ces formes demeure parcellaire. Notre objectif a été de les étudier en analysant la cohorte des patients français exposés à l'hGH. Les analyses épidémiologiques, ont montré, à partir de données quantifiées, pour la première fois chez l'homme, une relation entre la dose d'expos

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19

Gougerot, Alexianne. "Physiopathologie et thérapeutique des prions humains : une approche cellulaire." Thesis, Paris 6, 2017. http://www.theses.fr/2017PA066087/document.

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Les maladies à prions sont des pathologies neurodégénératives d’évolution fatale, transmissibles, pour lesquelles aucun traitement efficace n’existe. Elles associent sur le plan neuropathologique une spongiose, une gliose astrocytaire, une perte neuronale, et une accumulation de la forme anormalement repliée (PrPsc) de la protéine prion cellulaire codée par l’hôte. Certaines formes de cette maladie sont associées à une tauopathie et présentent des lésions neuropathologiques similaires à celles retrouvées dans la maladie d’Alzheimer (MA).Nous avons utilisé un modèle de cultures primaires de neu

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20

Ritchie, Diane Louise. "Characterisation of the agent strain in sporadic and variant Creutzfeldt-Jakob disease by transmission to wild-type mice." Thesis, University of Edinburgh, 2012. http://hdl.handle.net/1842/9563.

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Transmissible spongiform encephalopathy (TSE) strains are defined by their biological properties on transmission to wild-type mice, specifically by their characteristic incubation periods and patterns of vacuolar pathology (‘lesion profiles’) in the brain. Whilst a single TSE strain has been identified in variant Creutzfeldt-Jakob disease (vCJD), the phenotypic heterogeneity observed in sporadic CJD (sCJD) implies the existence of multiple strains of agent. These distinct strains are proposed to be enciphered by the different conformers of abnormal prion protein (PrP), recognised as different

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21

Briddell, Brian L. "Creutzfeld-Jakob disease : a synthesis of current literature for health care providers." Honors in the Major Thesis, University of Central Florida, 1998. http://digital.library.ucf.edu/cdm/ref/collection/ETH/id/23.

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This item is only available in print in the UCF Libraries. If this is your Honors Thesis, you can help us make it available online for use by researchers around the world by following the instructions on the distribution consent form at http://library.ucf.edu/Systems/DigitalInitiatives/DigitalCollections/InternetDistributionConsentAgreementForm.pdf You may also contact the project coordinator, Kerri Bottorff, at kerri.bottorff@ucf.edu for more information.<br>Bachelors<br>Health and Public Affairs<br>Nursing

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22

Harding, Mark R. "Analysis of post-mortem magnetic resonance image data of the human brain for the diagnosis of Creutzfeldt-Jakob disease." Thesis, University of Edinburgh, 2002. http://hdl.handle.net/1842/10934.

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Despite its generally low annual global incidence rate, Creutzfeldt-Jakob disease (CJD) has become a subject of considerable interest in recent years. This is due to concerns that there may be a possible causitive link with Bovine Spongiform Encephalopathy and that this may forewarn of a future epidemic of CJD in Humans. Despite recent discoveries of CJD related abnormalites in lymph-node tissues, in-vivo diagnosis of CJD is difficult and relies on a series of clinical tests and observations to provide a probabilistic diagnosis of the disease. The confirmed diagnosis is currently only obtainab

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23

McCormack, James E. "Characterisation of regulatory regions of the PRNP gene and their effect on susceptibility to sporadic and variant Creutzfeldt-Jakob Disease." Thesis, University of Edinburgh, 2001. http://hdl.handle.net/1842/24929.

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Based on analysis of PrP gene regulatory regions in other species, 4.8 Kb of human genomic sequence containing 1.5Kb of upstream sequence the first exon and 3.1Kb of intron was cloned from a human genomic lambda library into a CAT reporter gene. Transient transfections in human neuroblastoma cells of this construct and of nested deletions of this construct identified two regulatory regions, one between -43 and -9 bases upstream of the transcription initiation site and one within the intron between +292 and +622 basepairs. Having identified regulatory regions in neuroblastoma cells the effect o

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24

Klotz, Daniel Martin, and Rose Sarah Penfold. "Low mood, visual hallucinations, and falls – heralding the onset of rapidly progressive probable sporadic Creutzfeldt–Jakob disease in a 73-year old: a case report." Saechsische Landesbibliothek- Staats- und Universitaetsbibliothek Dresden, 2018. http://nbn-resolving.de/urn:nbn:de:bsz:14-qucosa-236920.

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Background Creutzfeldt–Jakob disease is a rare and rapidly fatal neurodegenerative disease. Since clinicians may see only very few cases during their professional career, it is important to be familiar with the clinical presentation and progression, to perform appropriate investigations, and allow for quick diagnosis. Case presentation A 73-year-old British Caucasian woman presented with acute confusion of 2 weeks’ duration on a background of low mood following a recent bereavement. Her symptoms included behavioral change, visual hallucinations, vertigo, and recent falls. She was mildly confu

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25

Barria, Matus Marcelo Alejandro. "Modelling human prion replication in cell-free systems." Thesis, University of Edinburgh, 2014. http://hdl.handle.net/1842/10025.

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One of the key molecular events in the transmissible spongiform encephalopathies or prion diseases is the conformational conversion of the cellular prion protein PrPC into the misfolded and pathogenic isoform, PrPSc. Prion diseases are fatal neurodegenerative conditions affecting humans and other animal species, which present with diverse clinical and neuropathological phenotypes. In humans, prion diseases can occur as sporadic, familial or acquired forms. Sporadic Creutzfeldt–Jakob disease (sCJD) accounts for the majority of cases. The current classification system of human prion diseases rec

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26

Aouba, Achille Eric. "Politiques et choix des produits plasmatiques ou recombinants en hémophilie : enjeux sécuritaires et déterminants institutionnels, médicaux et psycho-sociaux." Thesis, Sorbonne Paris Cité, 2015. http://www.theses.fr/2015PA05D004.

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Introduction et problématiques: Les politiques et choix des produits plasmatiques (PP) ou recombinants (PR) en hémophilie divergent selon les pays en dépit de recommandations internationales consensuelles. A la différence d'autres pays, la politique française en ce sens ne fait aucune priorité de choix entre les PP versus les PR en dépit de l'avènement de variante (v) de la maladie de Creutzfeldt-Jakob (MCJ) dont le risque transfusionnel affecté à ces premiers est jugé être infinitésimal. L'adhésion des soignants et des patients à cette politique ainsi que les répercussions psychologiques des

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27

GALENO, Roberta. "Characterization of human TSE strains after passage in humanized transgenic mice." Doctoral thesis, Università degli Studi di Verona, 2010. http://hdl.handle.net/11562/343521.

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Le encefalopatie spongiformi trasmissibili (EST) sono un gruppo di patologie neurodegenerative invariabilmente letali che si manifestano sia negli esseri umani che negli animali, causate da un agente trasmissibile la cui natura è in via di definizione. Ciononostante, le caratteristiche distintive di ciascun ceppo infettivo possono essere evidenziate e confrontate mediante passaggio sperimentale in una medesima specie di animali da laboratorio. Utilizzando questo approccio si è visto che il ceppo responsabile della variante di malattia di Creutzfeldt-Jakob (MCJv) umana è correlato direttamente

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Mantovani, S. "Modelli murini transgenici di malattie da prioni per lo studio del ruolo fisiopatologico della proteina prionica." Doctoral thesis, Università degli Studi di Milano, 2010. http://hdl.handle.net/2434/150216.

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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 polymorphism at codon 129 in the PrP gene. The most clear example of the phenotypic heterogeneity determined by the Met/Val polymorphism is represented by fatal familial insomnia (FFI) and Creutzfeldt-Jakob disease (CJD178), two clinically and neuropathologically distinct diseases linked to the D178N mutation

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29

Fagge, Timothy James. "A study of normal and abnormal forms of prion protein in the peripheral blood and tissues of patients with variant Creutzfeldt-Jakob disease." Thesis, University of Edinburgh, 2005. http://hdl.handle.net/1842/29751.

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This thesis assesses the potential use of PrP<sup>c</sup> as a surrogate marker for CJD by an analysis of blood from vCJD patients, sCJD patients, non-CJD neurological controls and healthy adults. PrP<sup>c</sup> was measured by DELFIA and cell-associated PrP was measured by flow cytometry. These are differences in free and cell-associated PrP found in blood of CJD patients and control groups, some of which may be useful as surrogate markers of disease DELFIA analysis identified a significant reduction in the concentration of PrP<sup>c</sup> in the whole blood of vCJD and non-CJD neurological

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30

Thüne, Katrin Verfasser], Inga [Akademischer Betreuer] [Zerr, Mikael [Gutachter] Simons, Silvio [Gutachter] Rizzoli, and Franc [Gutachter] Llorens. "Regional-dependent, comprehensive characterization of miRNA signatures in sporadic Creutzfeldt-Jakob Disease and early Alzheimer’s Disease-type neuropathology / Katrin Thüne ; Gutachter: Mikael Simons, Silvio Rizzoli, Franc Llorens ; Betreuer: Inga Zerr." Göttingen : Niedersächsische Staats- und Universitätsbibliothek Göttingen, 2019. http://d-nb.info/1196873984/34.

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Thüne, Katrin [Verfasser], Inga [Akademischer Betreuer] Zerr, Mikael [Gutachter] Simons, Silvio [Gutachter] Rizzoli, and Franc [Gutachter] Llorens. "Regional-dependent, comprehensive characterization of miRNA signatures in sporadic Creutzfeldt-Jakob Disease and early Alzheimer’s Disease-type neuropathology / Katrin Thüne ; Gutachter: Mikael Simons, Silvio Rizzoli, Franc Llorens ; Betreuer: Inga Zerr." Göttingen : Niedersächsische Staats- und Universitätsbibliothek Göttingen, 2019. http://d-nb.info/1196873984/34.

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Троцька, Ірина Олександрівна, Iryna Oleksandrivna Trotska та Ирина Александровна Троцкая. "Сучасні погляди на пріонові хвороби". Thesis, Видавництво СумДУ, 2008. http://essuir.sumdu.edu.ua/handle/123456789/3720.

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33

Baiardi, Simone <1985&gt. "Effect of host genotype and prion strain on the phenotypic heterogeneity of Creutzfeldt-Jakob disease: the peripheral nervous system involvement and the spectrum of the genetic form." Doctoral thesis, Alma Mater Studiorum - Università di Bologna, 2019. http://amsdottorato.unibo.it/9097/1/baiardi_simone_tesi.pdf.

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The first study was designed to assess whether the involvement of the peripheral nervous system (PNS) belongs to the phenotypic spectrum of sporadic Creutzfeldt-Jakob disease (sCJD). To this aim, we reviewed medical records of 117 sCJDVV2, 65 sCJDMV2K, and 121 sCJDMM(V)1 subjects for symptoms/signs and neurophysiological data. We looked for the presence of PrPSc in postmortem PNS samples from 14 subjects by western blotting and real-time quaking-induced conversion (RT-QuIC) assay. Seventy-five (41.2%) VV2-MV2K patients, but only 11 (9.1%) MM(V)1, had symptoms/signs suggestive of PNS involvemen

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Bishop, Matthew T. "Role of PRNP codon 129 genotype in defining strain transmission properties of human transmissible spongiform encephalopathy." Thesis, University of Edinburgh, 2009. http://hdl.handle.net/1842/4236.

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The human prion protein (PrP) gene (PRNP) codon 129 (M/V) polymorphism is a susceptibility factor for variant Creutzfeldt-Jakob Disease (vCJD) and a major determinant of clinico-pathological phenotype in sporadic CJD. The role of codon 129 in defining susceptibility and strain transmission properties has been investigated in three lines of transgenic mice that express human PrP. The human PRNP gene has directly replaced the murine version, by gene targeting, and variation at codon 129 has given the three genotype lines (HuMM, HuMV, and HuVV). The genetics of these three mouse lines are otherwi

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Gawinecka, Joanna Verfasser], Mathias [Akademischer Betreuer] [Bähr, Inga [Akademischer Betreuer] Zerr, Thomas [Akademischer Betreuer] Bayer, and Mikael [Akademischer Betreuer] Simons. "Prion protein-induced proteome alterations in sporadic Creutzfeldt-Jakob disease and in SH-SY5Y cell culture model / Joanna Gawinecka. Gutachter: Inga Zerr ; Thomas Bayer ; Mikael Simons. Betreuer: Mathias Bähr." Göttingen : Niedersächsische Staats- und Universitätsbibliothek Göttingen, 2011. http://d-nb.info/1042639671/34.

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DEL, GALLO FEDERICO. "SONNO E MALATTIE NEURODEGENERATIVE IN MODELLI ANIMALI." Doctoral thesis, Università degli Studi di Milano, 2014. http://hdl.handle.net/2434/232729.

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I disturbi del sonno rappresentano una manifestazione clinica tipica di gran parte delle malattie neurodegenerative. Insonnia, ipersonnia, parasonnie, perdita di specifiche fasi del sonno e disorganizzazione del ciclo veglia-sonno sono alcune delle manifestazioni che caratterizzano le sinucleinopatie, le prionopatie e le taupatie. Recenti evidenze hanno rilevato che in molti casi i disturbi del sonno possono precedere l’insorgenza del quadro sintomatico tipico di queste patologie. In particolare, è stato osservato che la presenza di RBD (disturbo del comportamento durante il sonno REM) nella s

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Tahir, Waqas Verfasser], Saima [Akademischer Betreuer] Zafar, Mikael [Gutachter] Simons, Michael [Gutachter] Hoppert, and Inga [Gutachter] [Zerr. "Identification and characterization of proteomic regulations in the cerebellum region of brain in MM1 and VV2 subtypes of sporadic Creutzfeldt-Jakob disease (sCJD) : Proteome Profiling of sCJD brain tissue / Waqas Tahir ; Gutachter: Mikael Simons, Michael Hoppert, Inga Zerr ; Betreuer: Saima Zafar." Göttingen : Niedersächsische Staats- und Universitätsbibliothek Göttingen, 2017. http://d-nb.info/1132336813/34.

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Soldevila, Trepat Marta. "Genetic variation in humans and chimpanzees in the prion protein gene." Doctoral thesis, Universitat Pompeu Fabra, 2005. http://hdl.handle.net/10803/7189.

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En el gen de la proteïna priònica, o PRNP, hem observat que el particular patró de variació que hem trobat basant-nos en dades de seqüenciació en humans es deu a selecció positiva, i que el mètode utilitzat per detectar selecció és crític. Utilitzant dades basades en SNPs es pot introduir un biaix al aplicar tests de neutralitat basats en diversitat de seqüències, i això pot portar a conclusions errònies. A més, hem vist que els polimorfismes en els codons 129 i 219 presenten gran diferències de freqüència en diferents poblacions humanes i també hem vist que aquestes posicions estan fixades en

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Kittner, Cornelia. "Risikofaktoren der sporadischen Creutzfeldt-Jakob-Krankheit." Doctoral thesis, 2008. http://hdl.handle.net/11858/00-1735-0000-0006-AF4A-9.

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Fincke, Fabian. "Doxycyclin bei der sporadischen Creutzfeldt-Jakob-Krankheit." Doctoral thesis, 2011. http://hdl.handle.net/11858/00-1735-0000-0006-B1C9-B.

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Mohanty, Siddhant. "Exploring potential drugs against Scrapie disease and Creutzfeldt-Jakob disease using molecular docking techniques." Thesis, 2014. http://ethesis.nitrkl.ac.in/6366/1/110BT0606-13.pdf.

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Scrapie is a lethal, degenerative sickness that influences the sensory systems of sheep and goats. Creutzfeldt-Jakob infection is a degenerative neurological issue that is serious and constantly lethal in human. Both the maladies are brought about by a protein called prion. The transformation of the á-helical, cell isoform of the prion protein (Prpc) to the insoluble, â-sheet rich irresistible, infection creating isoform (PrPsc) is the key occasion in prion illness. I have discovered two prion proteins on which the inhibitors could be docked. They are 1XYU (sheep) and 1FO7(human). I have addit

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Osmanlioglu, Seyma. "Steroid-responsive Enzephalopathie bei Autoimmunthyreoiditis als Differentialdiagnose der Creutzfeldt-Jakob-Krankheit." Doctoral thesis, 2016. http://hdl.handle.net/11858/00-1735-0000-0028-86F1-D.

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Gawinecka, Joanna. "Prion protein-induced proteome alterations in sporadic Creutzfeldt-Jakob disease and in SH-SY5Y cell culture model." Thesis, 2010. http://hdl.handle.net/11858/00-1735-0000-0006-ADDF-9.

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Thüne, Katrin. "Regional-dependent, comprehensive characterization of miRNA signatures in sporadic Creutzfeldt-Jakob Disease and early Alzheimer’s Disease-type neuropathology." Doctoral thesis, 2018. http://hdl.handle.net/21.11130/00-1735-0000-0005-126B-F.

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Kaune, Judith (Jansen). "Erstsymptom, Erstdiagnose und ärztlicher Erstkontakt bei Patienten mit sporadischer Creutzfeldt-Jakob-Krankheit in Deutschland." Doctoral thesis, 2014. http://hdl.handle.net/11858/00-1735-0000-0022-5E1C-4.

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Hintergrund: Die sporadische Creutzfeldt-Jakob-Krankheit (sCJK) ist eine seltene neurodegenerative Erkrankung. Bisher existieren nur wenige Studien im Hinblick auf das Initialstadium der Erkrankung. Ziel dieser Studie war es unter Berücksichtigung des M129V-Genotyps und des Prionproteintyps maximal zwei Erstsymptome begrenzt auf einen Zeitraum von 2 Wochen, den ärztlichen Erstkontakt und die erste fachärztliche Diagnose zu erfassen. Material und Methoden: In der vorliegenden Dissertation wurden retrospektiv Akten von 492 Patienten mit einer neuropathologisch gesicherten oder klinisch wahrschei

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Wohlhage, Marie Charlotte. "Entwicklung eines PrPc-Detektions-Assays zur Analyse der Fragestellung, welchen Einfluss PRNP-Mutationen oder Genpolymorphismen in CJK-Patienten auf die PrPc-Expression haben." Doctoral thesis, 2019. http://hdl.handle.net/21.11130/00-1735-0000-0003-C1BA-1.

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Tahir, Waqas. "Identification and characterization of proteomic regulations in the cerebellum region of brain in MM1 and VV2 subtypes of sporadic Creutzfeldt-Jakob disease (sCJD)." Doctoral thesis, 2016. http://hdl.handle.net/11858/00-1735-0000-0023-3E48-F.

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La maladie sporadique de Creutzfeldt-Jakob (sCJD) est une encéphalopathie spongiforme transmissible mortelle caractérisée par une large gamme de manifestations cliniques et pathologiques. Les caractéristiques pathologiques de la SDMC dépendent en grande partie de la présence d'une forme mal repliée de protéine prion cellulaire (PrPC); Connu sous le nom de PrPSC et polymorphisme (méthionine et valine) au codon 129 du gène PRNP qui code pour PrPC. Les facteurs étiologiques exacts de la MCSJ sont encore inconnus. Le génotype Codon 129 du gène PRNP et le type de PrPSC (type 1 ou type 2) influent s

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Krautwald, Lisa. "Klinische und diagnostische Eigenschaften der sporadischen Creutzfeldt-Jakob-Krankheit bei Patienten mit positiver Familienanamnese für Demenz oder Morbus Parkinson." Doctoral thesis, 2016. http://hdl.handle.net/11858/00-1735-0000-0028-8780-1.

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ZIEL Als Ursache für die sporadische Creutzfeldt-Jakob Krankheit wird eine spontane Konfigurationsänderung des Prionproteins diskutiert. Die Annahme der Beeinflussung fehlgefaltete Proteinketten, welche bei neurodegenerativen Erkrankungen wie der Alzheimer Demenz oder Parkinson vorliegen, auf die Entwicklung einer zweiten Proteinfehlfaltung stellen eine mögliche Verbindung zwischen dem Auftreten neurodegenerativer Erkrankungen und Prionerkrankungen her. Das Ziel dieser retrospektiven Untersuchung ist es, die klinischen und diagnostischen Eigenschaften von sCJD-Patienten mit Morbus Parkinson od

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Montag, Judith. "Übertragung von BSE auf nicht humane Primaten als Modell für die variante Creutzfeldt-Jakob Erkrankung (vCJD) im Menschen." Doctoral thesis, 2007. http://hdl.handle.net/11858/00-1735-0000-0006-AC5B-8.

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BONGIANNI, Matilde. "Detection of pathological prion protein by RT-QuIC analysis of cerebrospinal fluid and olfactory neuroepithelium of patients with sporadic Creutzfeldt-Jakob disease." Doctoral thesis, 2013. http://hdl.handle.net/11562/540350.

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ITALIAN SUMMARY PREMESSA: La diagnosi definitiva della forma sporadica di Creutzfeldt-Jakob (sCJD) in pazienti ancora in vita rimane una sfida. La Real time quaking-induced conversion (RT-QuIC) testata nel fluido cerebro- spinale (CSF) ha permesso l’identificazione di pazienti sCJD con una sensibilità del 80-90%. Poichè la CJD è una malattia trasmissibile, non trattabile e fatale, è importante effettuare una diagnosi corretta. Uno studio precedente ha individuato la presenza della proteina prionica patologica (PrP) nell’ epitelio olfattivo di pazienti sCJD. Questo studio ci ha spinto ha ve

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