|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Disease-causing mutations G93A and A4V show greatly altered reactivity patterns, which may contribute to the progression of familial ALS.
|
25096579 |
2014 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
We report that symptomatic male hSOD1(G93A) transgenic mice exhibit a deficiency in GH secretion similar to that seen in human ALS.
|
22621959 |
2012 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
These results suggest that enhanced glial TLR4 signalling during disease progression contributes to end-stage ALS pathology in hSOD1(G93A) mice.
|
25962427 |
2015 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
These are the first data that demonstrate an intrinsic hyperexcitability in the G93A cortical neurons due to a higher current density of the persistent sodium current in the mutated neurons and open up new prospects of understanding ALS disease etiopathology.
|
19071115 |
2009 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
We have previously shown that ALS-associated mutations in Cu/Zn superoxide dismutase 1 (SOD1) impair axonal transport of mitochondria in motor neurons isolated from SOD1 G93A transgenic mice and in ALS mutant SOD1 transfected cortical neurons, but the underlying mechanisms remained unresolved.
|
28973175 |
2017 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Actions of the antihistaminergic clemastine on presymptomatic SOD1-G93A mice ameliorate ALS disease progression.
|
27549088 |
2016 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Genetic biomarkers for ALS disease in transgenic SOD1(G93A) mice.
|
22412900 |
2012 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
The present study provides the first evidence that MnSOD immunoreactivity was increased in the central nervous system of SOD(G93A) transgenic mice, suggesting that mitochondria may play an important role in the pathogenesis and progress of ALS.
|
14568347 |
2003 |
|
Amyotrophic Lateral Sclerosis
|
|
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.
|
31020811 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Relationship between neuropathology and disease progression in the SOD1(G93A) ALS mouse.
|
21145892 |
2011 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
This finding highlights the utility of the SOD1-G93A transgenic mouse model for preclinical drug studies in ALS.
|
9419057 |
1997 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
We characterized synaptosomal glutamate transport activity in a recently developed transgenic rat model of amyotrophic lateral sclerosis (ALS) overexpressing the G93A Cu(2+)/Zn(2+) superoxide dismutase (SOD1) mutation.
|
12629173 |
2003 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
The release of [(3)H]D-aspartate ([(3)H]D-ASP) or [(3)H]GABA evoked by glycine from spinal cord synaptosomes was compared in mice expressing mutant human SOD1 with a Gly(93) Ala substitution ([SOD1-G93A(+)]), a transgenic model of amyotrophic lateral sclerosis, and in control mice.
|
12684256 |
2003 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Together, these findings strongly suggest that differential chaperone levels between skeletal muscle and spinal cord may be a critical determinant for G93A-induced protein misfolding in ALS.
|
23886956 |
2013 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Because peroxisome proliferator-activated receptor gamma (PPARgamma) agonists act as potent anti-inflammatory drugs, we tested whether superoxide dismutase (SOD1)-G93A transgenic mice, a mouse model of ALS, benefit from oral treatment with the PPARgamma agonist pioglitazone (Pio).
|
16120782 |
2005 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Effects of Ovariectomy in an hSOD1-G93A Transgenic Mouse Model of Amyotrophic Lateral Sclerosis (ALS).
|
29394243 |
2018 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Similar results were obtained from the reaction between familial ALS SOD mutants and H217O2: DMPO/.17OH (64%); DMPO/.16OH (36%) from A4V and DMPO/.17OH (62%); and DMPO/.16OH (38%) from G93A.
|
9618471 |
1998 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Metformin treatment has no beneficial effect in a dose-response survival study in the SOD1(G93A) mouse model of ALS and is harmful in female mice.
|
21909419 |
2011 |
|
Amyotrophic Lateral Sclerosis
|
|
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.
|
18316197 |
2008 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Our data indicate that the clinically relevant SOD1 G93A mutation, which leads to amyotrophic lateral sclerosis in humans and mice, does not necessarily worsen neuronal degeneration in other pathologies.
|
15350647 |
2004 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
In this study, for the first time, we adopted a method involving the intranasal administration of nerve growth factor combined with lateral ventricle NSC transplantation using G93A-SOD1 transgenic mice as experimental subjects to explore the treatment effect of this combined therapy in ALS.
|
28694091 |
2017 |
|
Amyotrophic Lateral Sclerosis
|
|
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).
|
29630712 |
2018 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
This study attempted to determine the effect of HUCB on SOD1 mice (transgenic B6SJL-TgN(SOD1-G93A)1GUR), which have a mutation of the human transgene, (CuZn superoxide dismutase gene SOD1) that has been associated with amyotrophic lateral sclerosis.
|
10896029 |
2000 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
In the present study, we evaluated the neuroprotective effects of 7,8-DHF in a transgenic ALS mouse model (SOD1(G93A)).
|
24637017 |
2014 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Longitudinal assessment of metal concentrations and copper isotope ratios in the G93A SOD1 mouse model of amyotrophic lateral sclerosis.
|
28067393 |
2017 |