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Showing 1 to 16 of 16 for “"TAR-DNA-binding protein"”.

  1. Exploring the Mitigation of TDP-43 Toxicity by Sis1 in Yeast

    … there is no cure. Research has revealed that Tar DNA-binding Protein 43 cytoplasmic aggregates are involved in many cases of Amyotrophic Lateral Sclerosis. One possible mechanism for TDP-43 induced toxicity is that these aggregates are titrating away a molecular chaperone protein, Sis1, from …

    unr Repository record for Exploring the Mitigation of TDP-43 Toxicity by Sis1 in Yeast (opens in a new tab)

  2. TDP-43 Is Directed to Stress Granules by Sorbitol, a Novel Physiological Osmotic and Oxidative Stressor

    TDP-43, or TAR DNA-binding protein 43, is a pathological marker of a spectrum of neurodegenerative disorders including amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U). TDP-43 is an RNA/DNA-binding protein implicated in …

    utswmed Repository record for TDP-43 Is Directed to Stress Granules by Sorbitol, a Novel Physiological Osmotic and Oxidative Stressor (opens in a new tab)

  3. Relationship Between TDP-43 Toxicity and Aggregation in Saccharomyces Cerevisiae

    <p>Protein aggregation and inclusion body formation are hallmarks of neurodegenerative diseases such as Alzheimer's, Parkinson's, Huntington's, and amyotrophic lateral sclerosis (ALS). These neurodegenerative diseases share a common pathology in that all include accumulation of insoluble protein

    denver Repository record for Relationship Between TDP-43 Toxicity and Aggregation in Saccharomyces Cerevisiae (opens in a new tab)

  4. Characterization of a Phosphomimetic Mutant of the ALS Associated Protein TDP-43

    <p>Trans-activation response (TAR) DNA-binding protein 43 (TDP-43) is a natively dimeric 414-residue protein that is encoded by the human <em>TARDBP</em> gene that has important implications in the pathogenesis of the neurodegenerative disorders ALS, FTD, and CTE. TDP-43 has been found …

    denver Repository record for Characterization of a Phosphomimetic Mutant of the ALS Associated Protein TDP-43 (opens in a new tab)

  5. A molecular analysis of the relation between TDP-43 and tau pathology

    Tau is a microtubule associated protein found in inclusions in tauopathies including Alzheimer's disease. One known cause of neurodegeneration is an excess of exon 10 inclusion in tau mRNA which is caused by several mutations in the MAPT gene, encoding tau. Processing of the Amyloid Precursor …

    kings Repository record for A molecular analysis of the relation between TDP-43 and tau pathology (opens in a new tab)

  6. Characterization of Codon Optimized Wild Type TDP-43 Mediated Neurodegeneration in a Drosophila Model for ALS.

    TAR DNA Binding 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 …

    utmb Repository record for Characterization of Codon Optimized Wild Type TDP-43 Mediated Neurodegeneration in a Drosophila Model for ALS. (opens in a new tab)

  7. Old-age hippocampal sclerosis in the aged population

    … it has been associated with both ischaemia and TAR-DNA-binding protein-43 (TDP-43)-related neurodegeneration. Variations in genes GRN, TMEM106B and ABCC9 are proposed as HS risk factors. The aim of this thesis was to investigate epidemiological, clinical, pathological and genetic characteristics …

    cambridge Repository record for Old-age hippocampal sclerosis in the aged population (opens in a new tab)

  8. Characterization of the Dimerization and Salt Dependent Aggregation of the ALS-Linked Protein TDP-43

    <p>Trans-active response (TAR) DNA-binding protein 43 (TDP-43) is essential for RNA processing but can also form toxic cytoplasmic inclusions in neurons of patients with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). RNA-binding has been shown to have the …

    denver Repository record for Characterization of the Dimerization and Salt Dependent Aggregation of the ALS-Linked Protein TDP-43 (opens in a new tab)

  9. Of Sex, Gut and Brain: Functional Studies in a Mouse Model of Amyotrophic Lateral Sclerosis-Frontotemporal Dementia

    … dimorphism is also evident 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 …

    calgary Repository record for Of Sex, Gut and Brain: Functional Studies in a Mouse Model of Amyotrophic Lateral Sclerosis-Frontotemporal Dementia (opens in a new tab)

  10. Identification and characterization of a pathological TDP-43 variant in amyotrophic lateral sclerosis and frontotemporal lobar degeneration

    TAR DNA-binding protein 43 (TDP-43) proteinopathy is a key pathological feature of a majority 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 …

    toronto-retro Repository record for Identification and characterization of a pathological TDP-43 variant in amyotrophic lateral sclerosis and frontotemporal lobar degeneration (opens in a new tab)

  11. TDP-43 and its role in neurodegeneration

    Ubiquitinated TDP-43 (TAR DNA Binding Protein) inclusions are a hallmark 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 …

    utmb Repository record for TDP-43 and its role in neurodegeneration (opens in a new tab)

  12. AGGREGATION MECHANISMS OF TDP-43 PROTEIN IN RESPONSE TO STRESS IN AMYOTROPHIC LATERAL SCLEROSIS AND THERAPEUTIC APPROACHES

    … present in 97% of ALS cases, consists in TDP- 43 proteinopathy characterized by the accumulation of ubiquitinated and phosphorylated TAR DNA-binding protein 43 (TDP-43) in the cytoplasm, accompanied by the concomitant loss of TDP-43 splicing activity in the nucleus of affected neurons. The …

    milano Repository record for AGGREGATION MECHANISMS OF TDP-43 PROTEIN IN RESPONSE TO STRESS IN AMYOTROPHIC LATERAL SCLEROSIS AND THERAPEUTIC APPROACHES (opens in a new tab)

  13. Dynamic changes of TDP-43 in testicular and epididymal sperm

    TAR DNA-binding protein of 43 kDa (TDP-43) is a ubiquitously expressed and evolutionarily conserved protein. TDP-43 is a DNA/RNA binding protein with several functions such as gene transcription, mRNA splicing and stability, transposon silencing, and micro RNA biogenesis. TDP-43 is associated with …

    uiuc Repository record for Dynamic changes of TDP-43 in testicular and epididymal sperm (opens in a new tab)

  14. THE INTERPLAY BETWEEN THE PROTEIN QUALITY CONTROL SYSTEM AND EXTRACELLULAR VESICLES IN THE DISPOSAL OF DISEASE-ASSOCIATED PROTEINS AND MIRNAS IN ALS AND FTD MODELS

    … inclusions containing the insoluble forms of the TAR DNA-binding protein of 43 KDa (TDP-43) and its C-terminal fragments (CTFs) of 35 (TDP-35) and 25 KDa (TDP-25). The accumulation of TDP-43 into insoluble cytoplasmic aggregates is toxic for cells that prevent their formation and/or promote their …

    milano Repository record for THE INTERPLAY BETWEEN THE PROTEIN QUALITY CONTROL SYSTEM AND EXTRACELLULAR VESICLES IN THE DISPOSAL OF DISEASE-ASSOCIATED PROTEINS AND MIRNAS IN ALS AND FTD MODELS (opens in a new tab)

  15. Commonalities between SMA and ALS: investigation of ribosome heterogeneity and translational defects

    … and their association with different proteins, known as ribosome-associated proteins (RAPs). In particular, these proteins can exert a direct role on mRNA selection and translation efficiency. At present, some RNA-binding proteins (RBPs), such as Fragile X Messenger Ribonucleoprotein 1 …

    trento Repository record for Commonalities between SMA and ALS: investigation of ribosome heterogeneity and translational defects (opens in a new tab)

  16. 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

    uiuc Repository record for CRISPR-based approaches for proteinopathies of the central nervous system (opens in a new tab)