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 “"FTLD"”.
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Behavioural disinhibition in the syndromes associated with frontotemporal lobar degeneration
… caused by frontotemporal lobar degeneration (FTLD) have highly heterogenous and overlapping features which complicate clinical and research practice. Behavioural impairments are associated with all FTLD syndromes, cause high morbidity and lack proven symptomatic treatments. Treatments for …
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Apathy and Impulsivity in Frontotemporal Lobar Degeneration
… Frontotemporal Lobar Degeneration (FTLD) is pathologically heterogeneous group of degenerative diseases of the brain. While there are distinct and highly recognisable clinical syndromes associated with FTLD there is also a wider and more diverse spectrum of progressive changes in …
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Identification and characterization of a pathological TDP-43 variant in amyotrophic lateral sclerosis and frontotemporal lobar degeneration
… (ALS) and frontotemporal lobar degeneration (FTLD) cases. One key feature of pathological TDP-43 is the presence of lower molecular weight (MW) C-terminal species of 25 and 35 kDa, the origins of which were unclear. In investigating the mechanism(s) which generate such species, we identified …
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Gain And Loss Of Progranulin Have Opposite Effects On Autophagy
… cause of frontotemporal lobar degeneration (FTLD), the most common type of dementia after Alzheimer’s disease (AD), whereas homozygous mutations result in neuronal ceroid lipofuscinosis (NCL), an infantile onset disease. During my Ph.D thesis, I found that hepatic PGRN overexpression in mice …
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Old-age hippocampal sclerosis in the aged population
… to frontotemporal dementia cases with TDP-43 (FTLD-TDP) caused by mutations in GRN, but differed from other FTLD-TDP subtypes. Results of this thesis reveal the importance of HS in the oldest old in the population, the key role of TDP-43, as well as providing robust methods to capture HS …
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A Study of alpha-Synuclein, Parkin and TDP-43 ; Proteins Implicated in Neurodegenerative Disease.
… patients with frontotemporal lobar degeneration (FTLD) and 25% of patients with Alzheimer's disease (AD) harbour TDP-43 immunoreactive pathological changes in their brains. Again, ELISAs were developed to investigate whether TDP-43 was present, or indeed increased amounts detected, in plasma. …
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TDP-43 Is Directed to Stress Granules by Sorbitol, a Novel Physiological Osmotic and Oxidative Stressor
… degeneration with ubiquitin-positive inclusions (FTLD-U). TDP-43 is an RNA/DNA-binding protein implicated in transcriptional and post-transcriptional regulation. Recent work also suggests that TDP-43 associates with cytoplasmic stress granules, which are transient structures that form in response …
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Relationship Between TDP-43 Toxicity and Aggregation in Saccharomyces Cerevisiae
… degeneration with ubiquitin-positive inclusions (FTLD-U). This thesis focuses upon the biophysical basis for TDP-43 aggregation in <em>S. cerevisiae</em>. Current <em>in vitro</em> evidence indicates that TDP-43 is a natively dimeric protein and that binding to RNA inhibits aggregation. …
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Neuropathological findings and staging in dementia
… in VaD and frontotemporal lobar degeneration (FTLD). Moreover, various neuropathological staging systems for AD differ in procedure and targeted pathology, and the choice of system affects the judgement of Alzheimer pathology and hence the final diagnosis. Also, various neuropathological LBD …
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Characterization of Codon Optimized Wild Type TDP-43 Mediated Neurodegeneration in a Drosophila Model for ALS.
… and frontotemporal lobar degeneration-ubiquitin (FTLD-U). The exact mechanism by which TDP-43 exerts toxicity in patient brains remains unclear. In a Drosophila model, we have identified robust gain of function phenotypes produced by misexpression of insect codon optimized wild type TDP-43 using …
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Investigating the aggregation of TDP-43 in models of Amyotrophic Lateral Sclerosis
… frontotemporal lobar degeneration (FTLD). Normally a nuclear protein, TDP-43 translocates to the cytoplasm and forms pathogenic inclusions in the disease-state, where the protein is often phosphorylated and cleaved and co-localises with stress granules. This thesis explores the …
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Overexpression of Heat Shock Factor 1 Protects against Pathological Proteins in Neurodegenerative Diseases
… is a pathological phenotype seen in ALS and FTLD, and my preliminary studies have shown that HSF1 induced a two-fold increase in nuclear TDP-43 in these double transgenic mice. These results have laid the framework for further research. This thesis provides new data supporting the therapeutic …
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Characterization of the Dimerization and Salt Dependent Aggregation of the ALS-Linked Protein TDP-43
… (ALS) and frontotemporal lobar degeneration (FTLD). RNA-binding has been shown to have the potential to decrease or inhibit the aggregation of TDP-43, indicating that supplying RNA therapeutics may be a solution to treat these neurodegenerative disorders. However, the mechanism of aggregation, …
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TDP-43 and its role in neurodegeneration
… of ALS (amyotrophic lateral sclerosis) and FTLD-TDP-43 (frontotemporal lobar degeneration with ubiquitin inclusions). These diseases share a similar pathology of cytoplasmic ubiquitinated TDP-43 inclusions, which contain C-terminally truncated TDP-43. Recent studies have shown that TDP-43 …
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Investigating the molecular environments and interactions of pathological TDP-43 filaments
… the gene encoding TDP-43, can cause ALS and FTLD by increasing TDP-43 filament formation, demonstrating a central role of TDP-43 pathology in disease. TDP-43 pathology starts in focal sites in the central nervous system (CNS) and spreads to connected regions over time, which correlates with …
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Development and utilization of Luminex biomarker assays for diagnosis and monitoring of neurodegenerative disease
… (DLB), fronto-temporal lobar degeneration (FTLD), motor neuron disease (MND), Huntington’s disease and the prion diseases. The incidence and prevalence of most of these diseases is rising, especially those that cause dementia, due to an increase in the average human life span. The diagnosis …
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Dynamic changes of TDP-43 in testicular and epididymal sperm
… (ALS) and frontotemporal lobar degeneration (FTLD). Our lab previously demonstrated that sperm from infertile men contains aberrant forms of TDP-43. We also characterized TDP-43 expression in the mouse testis. However, little is known about the functions of TDP-43 during sperm epididymal …
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Positron emission tomography in vivo characterisation of the pathology of frontotemporal dementia
… in vivo, through (i) [18F]AV-1451 imaging of the FTLD-tau pathology in a case of FTD due to a MAPT 10+16 mutation in the microtubule associated protein tau, and a second pre-symptomatic case with the same mutation; (ii) [18F]AV-1451 imaging of a cohort of seven cases with Semantic Dementia and one …