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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Novel behavioural characteristics of the superoxide dismutase 1 G93A (SOD1<sup>G93A</sup> ) mouse model of amyotrophic lateral sclerosis include sex-dependent phenotypes.
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31412164 |
2020 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Accumulation of DNA damage has been detected in the spinal cord of patients as well as in the G93A mouse model of amyotrophic lateral sclerosis (ALS).
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31676238 |
2020 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Compensatory changes in degenerating spinal motoneurons sustain functional sparing in the SOD1-G93A mouse model of amyotrophic lateral sclerosis.
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31364764 |
2020 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Globally we show a sex-specific benefit of dietary DHA supplementation in the G93A ALS mouse model, compared with mice fed an isocaloric control or a n-3-depleted diet.
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31755041 |
2020 |
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rs80265967
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0.100 |
GeneticVariation |
BEFREE |
Generation of an induced pluripotent stem cell line, ICGi014-A, by reprogramming peripheral blood mononuclear cells from a patient with homozygous D90A mutation in SOD1 causing Amyotrophic lateral sclerosis.
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31830646 |
2020 |
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rs121912436
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0.100 |
GeneticVariation |
BEFREE |
Our results showed that urate treatment provided neuroprotective effects as confirmed by enhanced survival, attenuated motor impairments, reduced oxidative damage and increased antioxidant defense in hSOD1-G85R-expressing Drosophila models of ALS.
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30690059 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Using a rodent model of ALS overexpressing mutant human Cu/Zn-superoxide dismutase gene (SOD1-G93A), we performed a comparative lipidomic analysis in motor cortex and spinal cord tissues of SOD1-G93A and WT rats at asymptomatic (~70 days) and symptomatic stages (~120 days).
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31406145 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
As a result, genistein alleviated ALS-related symptoms and slightly prolonged the lifespan of SOD1-G93A mice.
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31321663 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
In two different transgenic (Tg) mouse models of adult-onset neurodegenerative disease, a human A53T-α-synuclein (hαSyn) model of Parkinson's disease (PD) and a human G93A-superoxide dismutase-1(hSOD1) model of amyotrophic lateral sclerosis (ALS), mortality and survivor morbidity were significantly greater than non-Tg mice and a Tg mouse model of Alzheimer's disease after neonatal traumatic brain injury (TBI).
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31275228 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
In agreement, the hSOD1 G93A mouse model reveals that ALS-resistant CrMNs accumulate less insoluble hSOD1 and p62-containing inclusions than SpMNs.
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31157617 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
In humans, mutation of glycine 93 to alanine of Cu<sup>++</sup>/Zn<sup>++</sup> superoxide dismutase type-1 (SOD1-G93 A) has been associated to some familial cases of Amyotrophic Lateral Sclerosis (ALS).
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30503815 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Here, we used a similar longitudinal approach in the Cu/Zn superoxide dismutase (SOD1[G93A]) mouse model of ALS.
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30370671 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Hyperexcitability in Cultured Cortical Neuron Networks from the G93A-SOD1 Amyotrophic Lateral Sclerosis Model Mouse and its Molecular Correlates.
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31400485 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
In the present study, we investigated the effect of simvastatin on NSC34cells stably transfected with the G93A mutation in human SOD1 (NSC34-hSOD1G93A cells), a recognized in vitro model of ALS.
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31051215 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Evolution of the neurochemical profiles in the G93A-SOD1 mouse model of amyotrophic lateral sclerosis.
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29400109 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Recent studies have reported pathological abnormalities in oligodendrocytes in human patients with amyotrophic lateral sclerosis (ALS) and a mouse model of ALS expressing the G93A mutation of the human superoxide dismutase 1 (mtSOD1).
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31290185 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
In this study, we investigated oxidative stress in ALS model mice bearing both oxidative stress sensor nuclear erythroid 2-related factor 2 (Nrf2) and G93A-human Cu/Zn superoxide dismutase (Nrf2/G93A) treated by edaravone.
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30565312 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
(4) Conclusion: Besides the established histaminergic neuroprotective and anti-inflammatory effects, the induction of the heat shock response in the SOD1-G93A model by histamine confirms the importance of this pathway in the search for successful therapeutic solutions to treat ALS.
|
31382568 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Histidine treatment in SOD1-G93A mice proved broad efficacy in ameliorating ALS features, among which most importantly lifespan, motor performance, microgliosis, muscle atrophy, and motor neurons survival in vivo and in vitro.
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31020811 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
These results suggest that a deeper characterization of mechanisms involved in PACAP/EGFR/MMP-2 axis activation in G93A SOD1 mutated neurons may allow identifying new targets for ALS therapy.
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30238989 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
We identified a total of 10 studies; nine studies using cannabinoid treatment in transgenic SOD1-G93A ALS-model mice and one study in TDP-43 transgenic mice.
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30520038 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Furthermore, CPA resulted in an accelerated degeneration of motor neurons expressing human superoxide dismutase 1 (hSOD1) carrying the ALS-causing G93A mutation, compared to motor neurons expressing wild-type hSOD1.
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30446391 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
Overall our findings establish the significant impact of FM19G11-loaded NPs on the cellular pathways involved in self-renewal and proliferation in G93A-SOD1 epSPCs, thus providing an impetus to the design of novel tailored approaches to delay ALS disease progression.
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30909571 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
In the present study, we investigated hypoxic stress in ALS model mice bearing G93A-human Cu/Zn superoxide dismutase by in vivo HIF-1α imaging, and treated the ALS mice with DMOG.
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31344397 |
2019 |
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rs121912438
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0.100 |
GeneticVariation |
BEFREE |
In this study, we employed orthogonal cellular synchrotron radiation based spectro-microscopies to investigate the astrocytes of an ALS animal model: the rat hSOD1 G93A that overexpresses human mutated SOD1, which is known to increase the susceptibility of the SOD1 protein to form insoluble intracellular aggregates.
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30571081 |
2019 |