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 70 for “"SOD1"”.
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Exploring SOD1 electron transfer, heterodimerization, and hetero-amyloid formation.
Cu, Zn Superoxide dismutase (SOD1) is an essential metalloenzyme that regulates superoxide radicals. Because SOD1 is a long-lived protein, it contains an intrinsic molecular clock, deamidation, that accumulates over time. Misfolding and aggregation of SOD1 have been linked to neurodegenerative …
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Systematically and proactively testing variant effects for AIRE and SOD1
… 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 expectations and current …
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NEUROPROTECTIVE STUDIES ON THE MPTP AND SOD1 MOUSE MODELS OF NEURODEGENERATIVE DISEASES
The main, underlying cause of neurodegenerative disease is the progressive loss of neuronal structure or function, whereby central and/or peripheral nervous system circuitry is severely and irreversibly damaged, resulting in the manifestation of clinical symptoms and signs. Neurodegenerative …
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VDAC and SOD1: two major players in mitochondrial metabolism and in ALS
… Channel (VDAC) and the Superoxide Dismutase I (SOD1) and has been especially focused on the relationships between them in physiological or pathological conditions of the cell. VDAC is a pore-forming protein located in the outer mitochondrial membrane, where it is suspected to play a key role in …
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Recombinant DDX58 associates with SOD1 protein and selective miRNA changes in secreted EVs
… to ALS, as it was found upregulated in SOD1 and TDP-43 ALS models. Therefore, we decided to investigate DDX58 in connection to EVs and their paracrine effects. In NSC34 cells, we observed that recombinant DDX58-tGFP displays an intracellular distribution that partially reaches the …
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Caratterizzazione del ruolo della Superossido Dismutasi 1 (SOD1) in relazione al metabolismo mitocondriale
… descritte 3 isoforme diverse, la Cu-Zn SOD, o SOD1 è l isoforma più abbondante nel citosol. Negli ultimi anni l interesse verso lo studio di questa proteina è cresciuto sempre di più, dal momento che diverse mutazioni della proteina sono state ritrovate in tessuti di pazienti affetti da …
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Amyotrophic Lateral Sclerosis: An Exploration into the SOD1 Protein and a Representative Case Study
… 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 for a greater …
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Studying Aggregate Formation By Amyotrophic Lateral Sclerosis-Associated Mutant Sod1 Protein In Drosophila Model
… 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 goal of this project is to …
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MACROPHAGE RESPONSE TO INITIAL MUSCLE DENERVATION IN THE SOD1-G93A MOUSE MODEL OF AMYTROPHIC LATERAL SCLEROSIS.
… resistant (FR) and slow (S) NMJs. In the SOD1G93A mouse model of ALS, initial NMJ denervation occurs in the TA muscle (FF and FR fibers) between postnatal day (P)14 and P30, while little denervation occurs in soleus muscle (S and FR fibers) even late in disease. It is plausible that early …
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Understanding the gender-based mechanism of mso in als mice: a metabolic characterization of the sod1-g93a mouse model
… 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 that can become …
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Role of Pro-Apoptotic Bcl2-Homology-3 Domain (BH3)-Only Proteins in the Mutant SOD1 Mouse Model of ALS
… to mutations in Cu/Zn superoxide dismutase (SOD1), resulting in mitochondrial oxidative stress and intrinsic apoptosis. Transgenic mice expressing a G93A mutant of SOD1 provide an in vivo model to investigate motor neuron death during disease progression. The principal regulators of intrinsic …
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Electrostatic control of Cu, Zn superoxide dismutase aggregation in Amyotrophic Lateral Sclerosis : from lysine modification to interaction with lipid membranes.
Cu, Zn superoxide dismutase (SOD1) is a ubiquitous metalloprotein, which is responsible for protecting living cells from oxidative stress via disproportionation of superoxide ions. Misfolding and subsequent aggregation of SOD1 is casually linked to familial and sporadic cases of amyotrophic lateral …
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Overexpression of human Cu/Zn Superoxide Dismutase in Mice; The Effect of Increase Superoxide Scavenging on Autonomic Control of the Heart.
… endothelial tissues. Cu/Zn superoxide dismutase (SOD1) is an intracellular anti-oxidant enzyme that catalyzes dismutation of the superoxide anion (O2.-) to hydrogen peroxide (H2O2). Expression and function of this enzyme are diminished in pathologies that impair cardiovascular autonomic control. …
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Molecular Expression of Neuroprotective and Neurodestructive Signaling Systems Following Axotomy-Induced Target Disconnection: Relevance to ALS
… 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 pathological event is loss …
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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 copper in Parkinson's disease
… 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 substantia nigra contains …
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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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Protein Misfolding and Aggregation in Neurodegeneration: In Vitro And In Vivo Study Cases
… involved in ALS and AD. First, I looked at SOD1-G93A mutant protein, whose neuronal deposit is associated to familial and sporadic ALS. The mitochondrial porin VDAC1 has been proposed as a binding target of SOD1 mutant forms to mitochondria. By affinity studies we found that VDAC1 protein …
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A Novel Link Between Oxidative Stress Proteins and Estrogen Receptor Alpha-Mediated Gene Expression
… response including Cu/Zn superoxide dismutase (SOD1), thioredoxin (Trx) and thioredoxin reductase (TrxR). We found that SOD1 interacts with ERalpha, enhances ERalpha-ERE complex formation, influences estrogen responsiveness and associates with estrogen responsive regions of the pS2 and …
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QUANTIFYING OLIGOMERIC STATES OF PROTEINS IN-CELL VIA SINGLE-MOLECULE SUPER-RESOLUTION MICROSCOPY
… method. We investigated the dimerization of SOD1 by imaging SOD1-Halo-tag proteins conjugated with FRET donor fluorophore, PA-JF549, and acceptor fluorophore, JF646, in COS7 cells. Analyzing the FRET contribution on donor's intensity distributions allows us to estimate the dimerization of …
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