|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
We have reported that hSOD1-G93A transgenic mice modeling this disease show a more severe phenotype when the transgene is bred on a pure SJL background and a milder phenotype when bred on a pure B6 background and that these phenotype differences link to a region on mouse Chromosome 17.To examine whether other models of motor neuron degeneration are affected by genetic background, we bred the mutant human dynactin p150Glued (G59S-hDCTN1) transgene onto inbred SJL and B6 congenic lines.
|
25763819 |
2015 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
Our findings suggest iron as a key determinant of early motor neuron degeneration as well as proinflammatory responses at symptomatic stage in SOD1(G93A) mice.
|
26002422 |
2015 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
Here, we report the generation of transgenic pigs expressing mutant G93A hSOD1 and showing hind limb motor defects, which are germline transmissible, and motor neuron degeneration in dose- and age-dependent manners.
|
24577199 |
2014 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
The transgenic SOD1(G93A) mouse is a model of human amyotrophic lateral sclerosis (ALS) and recapitulates many of the pathological hallmarks observed in humans, including motor neuron degeneration in the brain and the spinal cord.
|
24239688 |
2014 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
We consider evidence that human mutant SOD1 protein in neonatal hSOD1(G93A) mice instigates motor neuron degeneration by increasing persistent sodium currents and excitability, in turn altering synaptic circuits that control excessive motor neuron firing and leads to excitotoxicity.
|
22740507 |
2012 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
Therefore, in the present study, we investigated the process of motor neuron degeneration as the disease progresses and to determine whether the CypA-AIF complex would play a role in inducing motor neuronal cell death in mutant superoxide dismutase 1 (SOD1)(G93A) ALS model mice.
|
20553309 |
2011 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
To establish the timeframe of motor neuron degeneration in relation to muscle atrophy in motor neuron disease, we have used MRI to monitor changes throughout disease in brain and skeletal muscle of G93A-SOD1 mice, a purported model of ALS.
|
21620832 |
2011 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
Acute glial activation by stab injuries does not lead to overt damage or motor neuron degeneration in the G93A mutant SOD1 rat model of amyotrophic lateral sclerosis.
|
20005223 |
2010 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
Taken together, this suggests that reduced levels of VGLUT2 decrease motor neuron degeneration but do not prevent loss of motor neuron function in the SOD1(G93A) mouse model for ALS.
|
19770042 |
2010 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
Therefore, the role of astrocyte proliferation was assessed in both acute and chronic mouse models of motor neuron degeneration, neuroadapted sindbis virus (NSV)-infected mice and SOD1(G93A) mice, respectively.
|
18410928 |
2008 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
To test whether ALS2 plays a protective role against mutant SOD1-mediated motor neuron degeneration in vivo, we examined the progression of motor neuron disease in SOD1(G93A) mice on an ALS2 null background.
|
16973244 |
2007 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
For this reason, we analyzed the temporal response of NPCs to motor neuron degeneration in the spinal cord and brain using nestin enhancer-driven LacZ reporter transgenic mice (pNes-Tg mice, control) and bi-transgenic mice containing both the nestin enhancer-driven LacZ reporter gene and mutant G93A-SOD1 gene (Bi-Tg mice).
|
17784831 |
2007 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
Results show that disruption of BBB and BSCB was evident in areas of motor neuron degeneration in G93A mice at both early and late stages of disease.
|
17512910 |
2007 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
In vivo quantification of spinal and bulbar motor neuron degeneration in the G93A-SOD1 transgenic mouse model of ALS by T2 relaxation time and apparent diffusion coefficient.
|
16740261 |
2006 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
While previous studies have demonstrated reductions in GLT1 with SOD1-mediated disease progression, it is not well established whether a reduction in this astrocyte-specific transporter alters the pathobiology of motor neuron degeneration in the SOD1(G93A) mouse.
|
16753145 |
2006 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
We report here that rats that express a human SOD1 transgene with two different ALS-associated mutations (G93A and H46R) develop striking motor neuron degeneration and paralysis.
|
16382787 |
2005 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
Transgenic mice overexpressing the human mutated form (G93A) of Cu,Zn-superoxide dismutase (mSOD1) develop motor neuron degeneration resembling amyotrophic lateral sclerosis.
|
14625013 |
2003 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
We analyzed the molecular mechanism underlying motor neuron degeneration in the transgenic mouse model expressing the SOD1 gene with G93A mutation.
|
11796754 |
2002 |
|
Motor neuron atrophy
|
|
0.100 |
GeneticVariation
|
BEFREE |
We report here that rats that express a human SOD1 transgene with two different ALS-associated mutations (G93A and H46R) develop striking motor neuron degeneration and paralysis.
|
11717358 |
2001 |