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 8 of 8 for “"TDP43"”.
-
RNA Dysregulation in a Novel Human Model of TDP43 Proteinopathy
… of ALS, over 97% of patients present with TDP43 pathology. This involves the nuclear-to-cytoplasmic mislocalisation and aggregation of TDP43, an important nuclear regulator of mRNA splicing. In this thesis, we describe a novel in vitro model allowing on-demand mislocalisation of endogenous …
-
Twist of messenger Fate: novel mechanisms for TDP43 in modulating mRNA decay and alternative polyadenylation
TDP43 is an ubiquitously expressed RNA-binding protein implicated in several aspects of RNA metabolism. It can shuttle between the nucleus and the cytoplasm; however, when it is mutated in some familial Amyotrophic Lateral Sclerosis (ALS) cases, it undergoes nuclear clearance and cytoplasmic …
-
Functional analysis of amyotrophic lateral sclerosis mutations using Drosophila cell culture models
… three key ALS-associated genes: FUS, SOD1, and TDP43, along with their respective three mutations (FUS: H517Q, R521C, P525L; SOD1: A4V, G85R, E100G; TDP43: G298S, Q331K, M337V) using Drosophila S2R+ cell models. This project combines molecular biology, high-throughput genetic screening, and …
-
Of Sex, Gut and Brain: Functional Studies in a Mouse Model of Amyotrophic Lateral Sclerosis-Frontotemporal Dementia
… in a mutant human TAR DNA binding protein 43 (TDP43) mouse model of ALS-FTD, whereby males have a much shorter lifespan than females. Interestingly, these mice exhibit an abnormally enlarged gut, suggesting an involvement of the gut microbiota (i.e., the collective microorganisms such as …
-
Commonalities between SMA and ALS: investigation of ribosome heterogeneity and translational defects
… and that the RBPs TAR DNA binding protein 43 (TDP43) and Matrin3 (MATR3), involved in ALS, are associated with the translation machinery. In addition, translatome changes are commonly observed in both diseases and loss of SMN and dysregulation of TDP43 and MATR3 are associated with translation …
-
Characterisation of Tau splicing factors in Alzheimer’s disease
… used was dependent on the presence or absence of TDP43 inclusions because approximately 30 % of AD cases have inclusions of the RNA binding protein, TDP-43 which may contribute to the clinical phenotypes observed in these cases. However TDP-43 is an RNA binding protein known to regulate …
-
Investigating pathological mechanisms in the Thy1-hTDP43 ALS mouse model
… to colocalise with an ALSassociated protein, TDP43, in spinal motor neurons, implying a pathological role for their formation. Additionally, key factors suppressed by protective SG formation were upregulated ROS and NLRP3, indicating altered SG function in ALS. Pathological stress granules are …
-
Positron emission tomography in vivo characterisation of the pathology of frontotemporal dementia
… of [18F]AV-1451 for different tau isoforms and TDP43-pathology, my analyses focus on multivariate distributions rather than absolute binding potential. The results show high correlations between [18F]AV-1451 and [11C]PK-11195 binding in each FTD syndrome. However, in the healthy MAPT 10+16 …