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 “"Frontotemporal lobar degeneration"”.
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Apathy and Impulsivity in Frontotemporal Lobar Degeneration
Apathy and Impulsivity in Frontotemporal Lobar Degeneration Ian Coyle-Gilchrist 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 …
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Apathy and Impulsivity in Frontotemporal Lobar Degeneration Syndromes
… pathological and genetic fractionation of frontotemporal lobar degeneration syndromes in recent years, driving the development of novel diagnostic criteria. However, phenotypic boundaries are not always distinct and syndromes converge with disease progression, limiting the insights …
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Behavioural disinhibition in the syndromes associated with frontotemporal lobar degeneration
The different clinical syndromes 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 …
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Imaging Correlates of Heterogeneity in the Syndromes Associated with Frontotemporal Lobar Degeneration
The syndromes associated with frontotemporal dementia are heterogeneous in their presentation and progression, with variable correlation between clinical phenotype and underlying proteinopathy. Single pathologies are associated with diverse clinical presentations, while the same clinical …
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Identification and characterization of a pathological TDP-43 variant in amyotrophic lateral sclerosis and frontotemporal lobar degeneration
… of amyotrophic lateral sclerosis (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 …
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TDP-43 Is Directed to Stress Granules by Sorbitol, a Novel Physiological Osmotic and Oxidative Stressor
… amyotrophic lateral sclerosis (ALS) and frontotemporal lobar 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 …
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Relationship Between TDP-43 Toxicity and Aggregation in Saccharomyces Cerevisiae
… inclusions of two of these diseases, ALS and frontotemporal lobar 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 …
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Neuropathological findings and staging in dementia
… the neuropathological findings. Furthermore, degeneration of the nucleus locus coeruleus, often seen already macroscopically, generally indicates the diagnoses Lewy body disease (LBD) or AD among the demented, while a preserved locus coeruleus occurs mainly in VaD and frontotemporal lobar …
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Characterization of Codon Optimized Wild Type TDP-43 Mediated Neurodegeneration in a Drosophila Model for ALS.
… Protein-43 (TDP-43) is known to mediate neurodegeneration associated with amyotrophic lateral sclerosis (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 …
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Gain And Loss Of Progranulin Have Opposite Effects On Autophagy
… PGRN is involved in both metabolism and neurodegeneration. It has recently emerged as an important regulatory adipokine of glucose metabolism and insulin sensitivity. Individuals with obesity and type 2 diabetes were shown to have increased serum PGRN levels. Heterozygous loss of function …
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Using induced pluripotent stem cells to model glial-neuronal interactions in TDP-43 proteinopathies
… disease protein in both ALS and a sub-group of frontotemporal lobar degeneration (FTLDTDP). Moreover, the identification of TARDBP mutations in familial ALS confirms a mechanistic link between the observed mis-accumulation of TDP-43 and neurodegeneration but also provides an opportunity to …
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The Discovery of p62-dependent Processing Bodies
… amyotrophic lateral sclerosis and <br/>frontotemporal lobar degeneration. This research found that during proteotoxic, endotoxic <br/>or oxidative stress, p62 bodies can transform into PBs through the sequential movement of <br/>RNA binding proteins into basal p62 bodies, thus …
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Investigating the aggregation of TDP-43 in models of Amyotrophic Lateral Sclerosis
… lateral sclerosis (ALS) and ubiquitin-positive 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 …
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Characterization of the Dimerization and Salt Dependent Aggregation of the ALS-Linked Protein TDP-43
… with amyotrophic lateral sclerosis (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. …
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TDP-43 and its role in neurodegeneration
… (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 binds to more …
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A Study of alpha-Synuclein, Parkin and TDP-43 ; Proteins Implicated in Neurodegenerative Disease.
… have shown that about 55% of 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 …
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Dynamic changes of TDP-43 in testicular and epididymal sperm
… amyotrophic lateral sclerosis (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 …
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Transdiagnostic approach to language and cognition in the logopenic variant of primary progressive aphasia and Alzheimer’s disease
… progressive aphasia (PPA), associated with frontotemporal lobar degeneration and Alzheimer’s disease (AD). Language deficits are also frequently found in other dementias like typical, amnestic AD. Progressive language deterioration negatively affects individuals’ quality of life, carer …