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 9 of 9 for “"motor neuron death"”.
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Role of Pro-Apoptotic Bcl2-Homology-3 Domain (BH3)-Only Proteins in the Mutant SOD1 Mouse Model of ALS
… disease involving rapid degeneration of motor neurons in the spinal cord and retraction of their axonal projections to the neuromuscular junctions. Several known mutations linked to some familial cases of ALS have been linked to mutations in Cu/Zn superoxide dismutase (SOD1), resulting in …
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Amyotrophic Lateral Sclerosis: mechanism behind mutant SOD toxicity and improving current therapeutic strategies
… Lateral Sclerosis (ALS) is an always lethal motor neuron disease with unknown pathogenesis. Inhibitors of the molecular chaperone heat shock protein 90 (Hsp90) have limited neuroprotection in some models of motor neuron degeneration. However the direct effect of Hsp90 inhibition on motor …
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Spinal Muscular Atrophy: Evidence of a Multi-System Disease
… disorder thought to be affecting primarily the motor neurons. As such, paralysis, motor weakness and death ensue. While SMA is most commonly seen in infants and children, it can span all ages. Its genetic etiology revolves around the homozygous deletion or mutation of the SMN1 gene, whose …
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Twist of messenger Fate: novel mechanisms for TDP43 in modulating mRNA decay and alternative polyadenylation
… clearance and cytoplasmic accumulation, driving neuronal degeneration. The same phenotype is present in patients bearing ALS-inducing mutations in other genes and ALS sporadic patients, defining TDP43 proteinopathy as a common feature in this pathology. Why does it cause specific motor neuron …
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Antioxidant Biomarkers and Nutraceutical Therapeutics in Neurodegeneration and Neurotrauma
… aggregation, axonal degeneration, and resulting neuronal death. These processes deplete the body’s endogenous antioxidant system. We report a retrospective analysis of antioxidant blood biomarkers in patients with a history of mTBI from a local sports medicine clinic, Resilience Code. We found …
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Identification and characterization of a pathological TDP-43 variant in amyotrophic lateral sclerosis and frontotemporal lobar degeneration
… of TDP-35 in cultured cells and primary motor neurons showed that TDP-35 has reduced solubility, cytoplasmic distribution, increased tendency to form aggregates, and the capacity to induce motor neuron death. Generation of a neo-epitope TDP-35 antibody immunolabeled the pathological 35 …
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Selective Neuronal Vulnerability in Neocortices from Patients with C9ORF72-related Neurodegeneration
… disorder classically characterised by motor neuron death. However, genetic and histopathological overlap with frontotemporal dementia (FTD) suggests extra-motor involvement, and that pathology likely extends to other neuronal populations (Chapter 1). This may be especially evident in …
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Understanding the gender-based mechanism of mso in als mice: a metabolic characterization of the sod1-g93a mouse model
… neurodegenerative disease characterized by motor neuron death and a corresponding loss of neuromuscular connections resulting in muscle atrophy. Patients become paralyzed shortly after symptom onset and typically die within one to five years of pulmonary complications. ALS is a relatively …
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The Apoptotic and Wallerian Degeneration Pathways Regulate Disease Onset and Progression in the SOD-1G93A Transgenic Mouse Model of ALS
… characterized by progressive muscle denervation, motor axon degeneration and the death of motor neurons. The molecular mechanisms that mediate axon degeneration in ALS remain unknown, but motor neuron cell body death occurs through apoptosis. Genetic deletion of the pro-apoptotic gene Bax delays …