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.
Results
Showing 1 to 20 of 28 for “"prion disease"”.
-
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 …
-
Deciphering the Molecular Basis of the Species Barrier in Prion Disease Using Bank Vole PrP
Prion disease is an infectious and fatal neurodegeneration condition which depends on the structural reconfiguration of a predominantly α-helical cellular protein called PrPC into a β-sheet rich conformer referred to as scrapie or PrPSc. The infectious nature of this conformer enables it to …
-
Molecular ecology and hierarchical models elucidate chronic wasting disease dynamics
Prions present a unique evolutionary scenario because a single gene codes for both a disease agent and a functionally constrained native protein. The prion precursor gene, Prnp, codes for the prion precursor protein, PrP, which is constitutively expressed as a native isoform within all mammals. …
-
Aggregation & Localization of a Disease-Associated Prion Protein (PrP) Mutant
… THE DISSERTATION Aggregation & Localization of a Disease-Associated Prion Protein: PrP) Mutant by Andrea Rhonda Zaragoza Medrano Doctor of Philosophy in Biology and Biomedical Sciences: Genetics) Washington University in St. Louis, 2009 Professor David A. Harris, Chairperson Prion protein: PrP) is …
-
Imaging of mice and men; adventures in multispectral imaging
… practically imaged intraoperatively to establish disease state. Secondly the redox state of haemoglobin is known to affect it's visible and near-infrared spectral characteristics. This project investigates whether it is possible to identify the haemodynamic response associated with functional …
-
Soil PH and clay content associated with chronic wasting disease in white-tailed deer in northern Illinois
… is an important reservoir for chronic wasting disease (CWD) which is a prion disease that infects cervids through both direct contact with infected animals and contact with contaminated environments. I built a boosted regression tree model that accurately predicted (AUC = 0.954) the probability …
-
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, …
-
The sHsp expression signature in the brain and modulation in models of chronic neurodegeneration
… expression<br/>under physiological (non-disease) conditions, they are also induced under conditions of<br/>stress/heat shock which is thought to play a role in response to protein misfolding that<br/>underpins disease. There are a wide range of diseases in which the sHsps function or …
-
Investigating The Dysregulation And Therapeutic Potential Of Neuroprotective Stress Response Proteins In Huntington’s Disease
Huntington’s disease (HD) is a fatal, genetic neurodegenerative disease that shares many features with other common neurological disorders, including early synapse loss. In both human HD brain and murine models, apoptotic pathways are dysregulated and mammalian target of rapamycin complex 1 …
-
Prions Propagate as Dynamic Strain Mixtures
Prions are the agent responsible for a group of transmissible and inevitably fatal neurodegenerative disease in humans and other mammals. Prion disease is caused by the misfolding of a host encoded protein, PrPC, into the infectious conformation PrPSc. Phenotypic differences in disease (i.e., …
-
Prion protein in health and disease
The prion protein (PrP) is a conserved glycoprotein tethered to cell membranes by a glycosylphosphatidylinositol anchor. In mammals, PrP is expressed in many tissues, most abundantly in brain, heart, and muscle. Importantly, PrP is required for prion diseases, which are neurodegenerative diseases …
-
Dilations of the Endoplasmic Reticulum Contribute to Spongiform Degeneration and Unlock a Door to a Unified Model of Neuropathological Features in Prion Diseases
Prion diseases are fatal neurodegenerative disorders characterized by spongiform degeneration, neuronal loss, and misfolded prion protein (PrPSc) deposition. The mechanistic origins of spongiform degeneration, which manifests as intracellular vacuolation in neurons, have remained unclear. We …
-
Modelling prion-induced neurodegeneration in PrP transgenic Drosophila
… of various genotypes to study the process of prion-induced neurodegeneration in this model. Prion diseases are caused by the occurrence of an abnormally-folded form of PrP (PrPSc) protein that arises either from the environment as an acquired disease, from mutation in the PrP-coding gene as a …
-
Biophysical methods to study the ubiquitination of amyloidogenic proteins and proteasomal responses to protein aggregates
… is a universal mechanism in neurodegenerative diseases such as Parkinson’s disease (PD), Alzheimer’s disease (AD) and prion disease. In mammalian cells, the majority of misfolded proteins are degraded by the ubiquitin-proteasome system (UPS), in which selective substrates are unfolded and …
-
The role of the cold shock protein, RBM3, in cooling, synaptic structural plasticity and neuroprotection
Neurodegenerative diseases are characterised by the accumulation of misfolded proteins and the progressive loss of synapses, leading eventually to neuronal demise. Synapse loss occurs early in disease and is a reversible process: the pruning and regeneration of synapses, known as structural …
-
Investigating the potential of enhanced neuroprotection through unfolded protein response inhibition and autophagy induction in neurodegeneration
Neurodegenerative diseases pose an immense challenge to the population and health care worldwide. There is a growing need for therapeutic strategies to target these diseases. Many neurodegenerative diseases are classified as protein misfolding diseases (PMDs). Despite their uniqueness, these PMDs …
-
Structural Elucidation of [RNQ+] prions Transmissible Infectivity
… of protein aggregation and understanding these diseases requires a large degree of knowledge pertaining to protein folding and misfolding. Neurodegenerative diseases associated with humans are commonly found to be the result of aggregated proteins or prions present in the brain. Prions are …
-
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. …
-
Deciphering Functional Significance of Substrate-Binding Domain of Ssa1 on Heat-Shock Response and Prion Propagation
… cellular functions such as heat shock response, prion propagation, protein folding and refolding, translocation across membranes and assembly of macromolecular complexes. A variety of evidence has accumulated to show that Hsp70 machinery is a key modulator of the stress response, such as heat …
-
Biochemical Studies of the ER Resident Kinase PERK and Implications for Local Translational Control
… has been shown to play an important role in disease pathology notably in neurodegenerative diseases. Increased levels of phosphorylated PERK and eIF2α have been observed in the brains of patients with Alzheimer’s disease, Parkinson’s disease, other tauopathies, and prion disease. In many of …
Page 1 of 2