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 “"Proteinopathies"”.
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CRISPR-based approaches for proteinopathies of the central nervous system
Submission published under a 24 month embargo labeled 'U of I Access', the embargo will last until 2026-05-01
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Using induced pluripotent stem cells to model glial-neuronal interactions in TDP-43 proteinopathies
… to recapitulate in vitro key aspects of TDP-43 proteinopathies such as MN cell autonomous toxicity and TDP-43 accumulation, but they can also be used to highlight previously unrecognised disease specific mechanisms and to test novel therapeutic approaches. Moreover, by performing co-culture …
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Evaluating suspected CWD feral pig brain samples using RT-QuIC and protocol for the purification and quality control of recombinant monomeric proteins for RT-QuIC assay
… and</p> <p>understood. The advances in other proteinopathies can then be experimentally applied to a</p> <p>specific disease of interest and the results compared to gain better understand the</p> <p>mechanisms underlying its pathogenesis and progression.</p> <p>Two examples of such …
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Disordered Protein Aggregates Are Linked to Changes in the Histone Post-Translational Modification Landscape in Disease and Non-Disease Models
… controlling misfolding by neurodegenerative proteinopathies. We believe that by studying the interface between neurodegeneration, protein misfolding, and epigenetics, we can elucidate the mechanisms leading to disease, and potentially reveal novel targets for therapeutic development. Our …
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TDP-43 Is Directed to Stress Granules by Sorbitol, a Novel Physiological Osmotic and Oxidative Stressor
… may contribute to the progression of TDP-43 proteinopathies.
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Characterization of a Phosphomimetic Mutant of the ALS Associated Protein TDP-43
… environmental stimulus for aggregation in TDP-43 proteinopathies. We expand upon the hypothesis that TDP-43 readily aggregates under agitation conditions and that the addition of poly-TG repeats to TDP-43 in aggregation conditions attenuates its aggregation propensity. We expressed a recombinant …
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Multi-omic Analysis of Neurodegeneration in Alzheimer’s Disease and Related Dementias
… at the single cell resolution of the following proteinopathies: Alzheimer’s Disease (AD), Frontotemporal Dementia (FTD), Lewy Body Dementia (LBD), and Vascular Contributions to Cognitive Impairment and Dementia (VCID). We utilize both single-cell RNA sequencing (scRNA-seq) and single-cell ATAC …
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Serpin polymers enforce molecular filtration in the endoplasmic reticulum
… provides a template for understanding related proteinopathies as diverse as an autosomal dominant form of dementia and diabetes insipidus, and identifies ER quality control components as potential therapeutic targets.
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Characterization of Codon Optimized Wild Type TDP-43 Mediated Neurodegeneration in a Drosophila Model for ALS.
… understand the exact disease mechanism of TDP-43 proteinopathies.
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On the reaction coordinate optimality for reversible aggregation kinetics
… science to the treatment and prevention of proteinopathies in the biological sciences. Here we are particularly interested in aggregation processes in intrinsically finite macromolecular systems, such as colloidal suspensions and protein solutions. These systems undergo transitions between …
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RNA Dysregulation in a Novel Human Model of TDP43 Proteinopathy
… tool for studying the wide spectrum of TDP43 proteinopathies, and act as a crucial platform for the development of therapeutics for the treatment of ALS.<p></p>
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Biophysical and Therapeutic Approaches to Neurodegenerative Disease: Insights From Lysosomal and RNA-Binding Protein Systems
… how lysosomal storage disorders and RNA‑binding proteinopathies exemplify convergent routes to neurodegeneration and motivate a cross‑disease, mechanistically grounded therapeutic perspective. I also examined the cellular fate of TPP1 from its synthesis in the endoplasmic reticulum to its …
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Selective Degradation of Aggregated Tau Protein Via Vectored Nanobody-TRIM21 RING Fusion Constructs
… but for the various other neurodegenerative proteinopathies driven by pathological protein aggregation.
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Overexpression of Heat Shock Factor 1 Protects against Pathological Proteins in Neurodegenerative Diseases
… HSF1 levels for the treatment of TDP-43 proteinopathies and ALS.
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The role of mTOR signaling in Alzheimer s disease
… a process that is altered in AD and other proteinopathies. Another known function of mTOR signaling is the regulation of synaptic plasticity and function. Using two widely used animal models of AD, known as Tg2576 and 3xTg-AD mice, we employed multidisciplinary approaches to dissect the …
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The sHsp expression signature in the brain and modulation in models of chronic neurodegeneration
… of intracellular (R6/2) and extracellular (ME7) proteinopathies. These<br/>models recapitulate key features of Huntington’s and prion disease, respectively.<br/><br/>Analysis of the sHsps in the R6/2 Huntington’s disease (HD) mouse model showed a specific<br/>down-regulation of HspB5 in the white …
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DESIGN, SYNTHESIS AND BIOLOGICAL PROFILING OF NEW COMPOUNDS: INFECTIOUS AND NEURODEGENERATIVE DISEASES AS TARGETS
… I immersed myself in the captivating realm of proteinopathies, dedicating my efforts to the synthesis and characterization of innovative neuroprotective compounds aimed at combatting the devastating neuropathologies stemming from misfolded proteins. This ambitious endeavor commenced with a …
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THE ROLE OF HSF1 PROTEIN REGULATION ON NEURODEGENERATION
Cellular protein homeostasis is achieved by a delicate network of molecular chaperones and various proteolytic processes such as ubiquitin–proteasome system (UPS) to avoid a build-up of misfolded protein aggregates. The latter is a common denominator of neurodegeneration. Neurons are found to be …