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 20 of 21 for “"Superoxide dismutase 1 (SOD1)"”.
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Amyotrophic Lateral Sclerosis: An Exploration into the SOD1 Protein and a Representative Case Study
… research is making headway into understanding superoxide dismutase 1 (SOD1), an important antioxidant that is heavily implicated in the pathogenesis of familial ALS. The goals of this thesis are to help understand the molecular basis of SOD1 in relation to ALS and to demonstrate the dire need …
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Unraveling the molecular mechanism underlying ALS-linked astrocyte toxicity for motor neurons
Mutations in superoxide dismutase-1 (SOD1) cause a familial form of amyotrophic lateral sclerosis (ALS), a fatal paralytic disorder. Transgenic mutant SOD1 rodents capture the hallmarks of this disease, which is characterized by a progressive loss of motor neurons. Studies in chimeric and …
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Systematically and proactively testing variant effects for AIRE and SOD1
… polyendocrine syndrome type 1 (APS-1); and 2) superoxide dismutase 1 (SOD1), which is implicated in amyotrophic lateral sclerosis (ALS). The resulting variant effect map of AIRE missense variants, using an insulin promoter-driven reporter assay, agrees with previously reported biochemical …
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A Role for BDNF-Trkb Signaling in the Modulation of Superoxide Dismutase-1 Expression
Superoxide dismutase-1 (SOD1) has been implicated in the pathogenesis of familial amyotrophic lateral sclerosis (fALS), a degenerative motor neuron disease more commonly known as Lou Gehrig’s disease. The mechanism by which it causes degeneration and the extent of its involvement are currently …
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The role of L-carnitine in preventing mitochondrial dysfunction after neonatal hypoxia-ischemia
… before and after OGD. We observed a decrease in superoxide and H2O2 in RHSC treated with LCAR and exposed to OGD. In further experiments we observed LCAR treatment increased the expression and activity of superoxide dismutase 1 (SOD1) and catalase prior to OGD and this effect resulted in …
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The role of L-carnitine in preventing mitochondrial dysfunction after neonatal hypoxia-ischemia
… before and after OGD. We observed a decrease in superoxide and H2O2 in RHSC treated with LCAR and exposed to OGD. In further experiments we observed LCAR treatment increased the expression and activity of superoxide dismutase 1 (SOD1) and catalase prior to OGD and this effect resulted in …
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Molecular Expression of Neuroprotective and Neurodestructive Signaling Systems Following Axotomy-Induced Target Disconnection: Relevance to ALS
… of familial cases with a mutation in the gene superoxide dismutase 1 (SOD1) gene led to the development of a transgenic mouse model. Pre-symptomatic SOD1 mice show no symptoms well into adulthood, however once symptom onset has occurred they display pathological hallmarks of ALS. The initial …
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Overexpression of Heat Shock Factor 1 Protects against Pathological Proteins in Neurodegenerative Diseases
… such as TAR DNA/RNA binding protein (TDP-43) and superoxide dismutase 1 (SOD1), which lead to neuronal dysfunction and loss. Therefore, molecules that maintain protein homeostasis may be beneficial therapeutic targets to correct the imbalance in protein folding and clearance in these disorders. …
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Feeding Female Growing Rats Different Sources of Omega-3 Polyunsaturated Fatty Acids Affects Lung Fatty Acid Composition and Gene Expression
… (CAT), glutathione peroxidase (GSH-Px) and superoxide dismutase 1 (SOD1), were determined using real-time polymerase chain reaction.;Results: Rats fed FO, with the highest alpha-linolenic acid (ALA, 18:2n-3) content, had the highest (P<0.001) lung ALA deposition. Rats fed KO, with the …
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The role of copper in Parkinson's disease
… the function of the antioxidant cuproprotein, superoxide dismutase 1 (SOD1). Characterisation of regional copper levels, and copper transport pathways in the normal human brain using inductively coupled plasma-mass spectrometry (ICP-MS), western blot and immunohistochemistry demonstrated the …
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Computational Modeling and Analysis of Protein Interactions at Multiple Coarse-grained Levels in Biological Systems
… specific research questions. We focus on the Superoxide Dismutase 1 (SOD1) protein, which can cause aggregation and Amyotrophic Lateral Sclerosis (ALS) when mutated. Despite numerous studies on SOD1 folding, understanding the interplay between crowding, folding and aggregation in vivo remains …
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The genetic and biochemical analysis of Drosophila Wwox protein function.
… consistent changes in different isoforms of superoxide dismutase 1 (Sod1) were identified. Due to the known roles these proteins play in pro and anti-apoptotic pathways, it is possible that Sod1 and Wwox may work in concert to regulate the delicate balance of defence mechanisms in response to …
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Studying Aggregate Formation By Amyotrophic Lateral Sclerosis-Associated Mutant Sod1 Protein In Drosophila Model
… of ALS cases are sporadic, however, mutations in superoxide dismutase 1 (SOD1) are responsible for about 20% of familial ALS (fALS). Mutated SOD1 proteins are prone to misfold and form protein aggregates, thus representing a good candidate for studying aggregate formation.</p> <p>The long-term …
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VOLTAGE GATED SODIUM CHANNEL AND NEURONAL DIFFERENTIATION. IMPLICATIONS IN AMYOTROPHIC LATERAL SCLEROSIS PATHOGENESIS
… that express the mutant G93R form of the superoxide dismutase 1 (SOD1) enzyme, show that alteration in the INaP of spinal inter- and moto- neurons induced neuron hyperexcitability that finally leads to hyperactive zebrafish locomotor behaviour together with morphological alterations of …
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Identification and Characterization of Factors Involved in Sodium Selenite Toxicity
… that the SeL toxicity can be modulated by Cu/Zn superoxide dismutase 1 (Sod1) activity. This observation was surprising since Sod1 is one of the most important antioxidant enzymes and is needed for ROS tolerance. Interestingly, substitution of Sod1 by the E. coli Fe SodB enzyme suppressed SeL …
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New Therapeutic Strategies for Amyotrophic Lateral Sclerosis Treatment
… of those are dominantly inherited mutations in superoxide dismutase 1 (SOD1). The transgenic animal model of ALS was the G93A mouse that expresses a mutant form of human SOD1 carrying the Gly93 → Ala and develops motor neuron disease similar to familial ALS. With the lack of effective drug …
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Investigating the role of mitochondrial dysfunction in a Drosophila model of C9orf72 ALS/FTD
… of antioxidants such as mitochondrial superoxide dismutase 2 (SOD2) and catalase were able to rescue impaired larval locomotion. Surprisingly, overexpression of cytosolic superoxide dismutase 1 (SOD1) exacerbated larval crawling phenotypes. Together, these data suggest a causative link …
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Understanding the gender-based mechanism of mso in als mice: a metabolic characterization of the sod1-g93a mouse model
… with both forms of ALS, including mutations in superoxide dismutase 1 (SOD1), TDP-43, and FUS, as well as several other genes that have yet to be identified. These genes encode a variety of proteins that encompass several processes within the cell, leading to an array of molecular mechanisms …
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TUMOR PROMOTING FUNCTIONS FOR THE METABOLIC REGULATOR SIRT5
… dehydrogenase (G6PD) and desuccinylating superoxide dismutase 1 (SOD1). Recent studies revealed that SIRT5 knock-down in human breast and lung cancer cells inhibited their transformed properties, whereas it had no significant effect on the growth of normal cells. Furthermore, it has been …
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