SOD1 protein
SOD1 protein aggregates stimulate macropinocytosis in neurons to facilitate their propagation
... G93A SOD1 were expressed and purified from ...56]. SOD1 aggregation was performed in vitro as previously described ...mutant SOD1 protein (1 mg/mL) in PBS were co-incubated with 20 mM ...
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Features of wild-type human SOD1 limit interactions with misfolded aggregates of mouse G86R Sod1
... fluorescent protein (GFP) have demonstrated normal dimerization and full activity of the SOD1 protein, and thus fusion of SOD1 with such fluorescent reporters does not necessarily alter the ...
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ALS-linked misfolded SOD1 species have divergent impacts on mitochondria
... the SOD1 protein, resulting in a toxic gain of ...misfolded SOD1 protein, and have been used as therapeutics in pre-clinical ...Misfolded SOD1 selectively associates with spinal cord ...
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SOD1 aggregation in astrocytes following ischemia/reperfusion injury: a role of NO-mediated S-nitrosylation of protein disulfide isomerase (PDI)
... ubiquitinated protein, and colocalization of ubiquitin with SOD1 protein Since the monoclonal anti-ubiquitin antibody can recognize both free and conjugated ubiquitin, it was used to monitor both ...
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Overexpression of human SOD1 improves survival of mice susceptible to endotoxic shock
... of SOD1 in the mixed-genetic-background mice (tg-JAX) did not improve the survival ...the SOD1 protein is placed is important when evaluating the effectiveness of scavenging superoxide anions on ...
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Conformational specificity of the C4F6 SOD1 antibody; low frequency of reactivity in sporadic ALS cases
... of SOD1 protein could reveal the epitope re- quired for C4F6 ...the protein crosslinks formed from fixing the tissue with para- formaldehyde followed by an overnight incubation in 6 M GdnHCl to ...
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In vivo kinetic approach reveals slow SOD1 turnover in the CNS
... in SOD1 cause dominantly inherited ALS via a toxic gain of ...against SOD1, a gene responsible for 20% of familial ALS cases, has shown great promise in animal models and has recently completed a phase I ...
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An endogenous peptide marker differentiates SOD1 stability and facilitates pharmacodynamic monitoring in SOD1 amyotrophic lateral sclerosis
... intracellular SOD1 degradation described in this manuscript pro- vides a simple and highly sensitive assay, using CSF, for assessing SOD1 protein stability and degradation in ...greater ...
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The Effect of SOD1 Mutation on Cellular Bioenergetic Profile and Viability in Response to Oxidative Stress and Influence of Mutation-Type.
... the SOD1 protein increases metabolic functional susceptibility to oxidative ...mutant SOD1 transgenes (G93A, G37R, H48Q) to investigate the impact of oxidative stress on cell viability and metabolic ...
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Differential induction of mutant SOD1 misfolding and aggregation by tau and α-synuclein pathology
... of SOD1 fused to YFP as a reporter in a paradigm akin to the foregoing ...of SOD1 fused to YFP as model in studies of prion-like propaga- tion of misfolded SOD1 ...G85R- SOD1:YFP [46]. Thus, ...
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Targeting SOD1 induces synthetic lethal killing in BLM- and CHEK2 -deficient colorectal cancer cells
... between SOD1 and both BLM and CHEK2 in two distinct cell ...that SOD1 silencing and inhibition (ATTM and LCS-1 treatments), or the induction of reactive oxygen species (2ME2 treatment) induces selective ...
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SOD1 mutations disrupt redox sensitive Rac regulation of NADPH oxidase in a familial ALS model
... for SOD1 and ...bovine SOD1 followed by Western blot for SOD1, Rac1, and ...bovine SOD1 followed by Western blot for SOD1 and ...
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Randomized, double-blind, placebo-controlled trial of arimoclomol in rapidly progressive SOD1 ALS
... An important limitation of this trial is the small sample size, which was a function of the ultrarare population targeted for enrollment. We estimate that at most 320 patients across the entire United States could have ...
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Actions of the antihistaminergic clemastine on presymptomatic SOD1-G93A mice ameliorate ALS disease progression
... of SOD1 mutant mice [7] and autophagy overexpression is proved in motor neurons of ALS mice by in vivo optical imaging [8]; moreover, inefficient autophagosome- lysosome fusion is reported in ALS patients [9, 10] ...
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Original Article miRNA-9 expression is upregulated in the spinal cord of G93A-SOD1 transgenic mice
... paired [13]. miRNAs also have an important function in the pathogenesis of neurodegener- ative diseases such as Alzheimer’s [14], Parkinson’s [15], and Huntington’s diseases [16]. In ALS, a few studies on miRNAs have ...
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Disrupting SOD1 activity inhibits cell growth and enhances lipid accumulation in nasopharyngeal carcinoma
... of SOD1 on lipid metabolic enzymes ...CPT1A protein level in the cells treated with LCS-1 or transfected with SOD1 siRNA decreased ...of SOD1 in- creased CPT1A protein levels ...the ...
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Lipopolysaccharide Modified Liposomes for Amyotropic Lateral Sclerosis Therapy: Efficacy in SOD1 Mouse Model
... cell loss after induced hypoxic injury in gerbils and rats [9,10]. Subsequent research with minocycline in various models of acute and chronic neurodegeneration corrobo- rated its neuroprotective effect, and extended ...
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Effect of thymic stimulation of CD4+ T cell expansion on disease onset and progression in mutant SOD1 mice
... male SOD1 G93A mice with an ana- bolic steroid, nandrolone decanoate, worsened clinical outcome, although castration alone did not significantly modify survival ...male SOD1 G93A mice promoted muscle growth ...
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Stathmin 1/2-triggered microtubule loss mediates Golgi fragmentation in mutant SOD1 motor neurons
... twice. Data were analysed with Microsoft Excel or Graph- Pad Prism (GraphPad). Data from two groups showing each Gaussian distribution were analysed with student's t-test; otherwise the Mann–Whitney U test was used. Data ...
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Preliminary Observation about Alteration of Proteins and Their Potential Functions in Spinal Cord of SOD1 G93A Transgenic Mice
... Finally, it was questionable that we only used one mouse per group, it seen to not accord with the statistically routine requirement. But despite for all that, it didn’t bring the important effect for the statistically ...
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