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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
Our study comprehensively described the details of the possible abnormal proteins participated in the pathogenesis of SOD1 G93A transgenic mice, extensively explored their possible molecular mechanisms how to play the role in the development in this animal model, and provided some evidences and clues for further and deeply studying the relationship between the abnormal proteins and the pathogenesis of ALS in the other animal models and ALS patients.
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30123078 |
2018 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
BNIP1 expression was significantly reduced in spinal cord motor neurons from patients with ALS (4 controls: mean age, 60.5 years, mean [SE] value, 3984 [760.8] arbitrary units [AU]; 7 patients with ALS: mean age, 56 years, mean [SE] value, 1999 [274.1] AU; P = .02), in an ALS mouse model (mean [SE] value, 13.75 [0.09] AU for 2 SOD1 WT mice and 11.45 [0.03] AU for 2 SOD1 G93A mice; P = .002) and in brains of patients with PSP (80 controls: 39 females; mean age, 82 years, mean [SE] value, 6.8 [0.2] AU; 84 patients with PSP: 33 females, mean age 74 years, mean [SE] value, 6.8 [0.1] AU; β = -0.19; P = .009) or FTD (11 controls: 4 females; mean age, 67 years; mean [SE] value, 6.74 [0.05] AU; 17 patients with FTD: 10 females; mean age, 69 years; mean [SE] value, 6.53 [0.04] AU; P = .005).
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29630712 |
2018 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
Specifically, we report that expression of the mitochondrial isoform of SIRT3 is altered in muscle from the G93A-SOD1 mice during progression of disease; this alteration influences mitochondrial metabolism, which may be relevant for the well known energetic alterations taking place in ALS patients.
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28449871 |
2017 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
As proof of concept, we use the IS paradigm to express murine Interleukin (IL)-10 in the spinal cord of the SOD1-G93A transgenic mouse model of amyotrophic lateral sclerosis.
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25228069 |
2015 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
To eliminate these two sources of heterogeneity we have characterized plasma amino acids and other metabolites in the SOD1(G93A) transgenic mouse model for ALS.
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24129262 |
2014 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
A major QTL on mouse chromosome 17 resulting in lifespan variability in SOD1-G93A transgenic mouse models of amyotrophic lateral sclerosis.
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25008789 |
2014 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
Moreover, these results may pave the path for using the mSOD1(G93A) mouse model and these biomarkers as molecular beacons to evaluate the effects of novel drugs/treatments in ALS.
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23836781 |
2013 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
In the SOD1 Gly93Ala rat model of amyotrophic lateral sclerosis, the antisense oligonucleotide ISIS 333611 delivered to CSF decreased SOD1 mRNA and protein concentrations in spinal cord tissue and prolonged survival.
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23541756 |
2013 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
While there are legitimate concerns about the physiological differences between the rodent and human motor systems, mice expressing the 'G93A' superoxide dismutase-1 gene mutation are a predictable and robustly-characterized model for amyotrophic lateral sclerosis (ALS).
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22117132 |
2012 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
Progressive motor unit loss in the G93A mouse model of amyotrophic lateral sclerosis is unaffected by gender.
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19208415 |
2009 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
Tissue inhibitor of metalloproteinases-3 (TIMP-3) expression is increased during serum deprivation-induced neuronal apoptosis in vitro and in the G93A mouse model of amyotrophic lateral sclerosis: a potential modulator of Fas-mediated apoptosis.
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18316197 |
2008 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
In the present report we used the G93A transgenic mouse model of amyotrophic lateral sclerosis to develop and characterize an in vitro tool for the investigation of subtle alterations of spinal tissue prior to frank neuronal degeneration.
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16442737 |
2006 |
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rs1475170339
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0.100 |
GeneticVariation |
BEFREE |
Temporal patterns of cytokine and apoptosis-related gene expression in spinal cords of the G93A-SOD1 mouse model of amyotrophic lateral sclerosis.
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12124437 |
2002 |