|
Motor Neuron Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
Using a mouse model expressing a mutant SEMA3A with diminished signaling capacity, we studied the influence of SEMA3A signaling at the NMJ with two denervation paradigms; a motor neuron disease model (the G93A-hSOD1 ALS mouse line) and an injury model (BotoxA-induced paralysis).
|
28103314 |
2017 |
|
Motor Neuron Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
Here, to investigate the role of proliferating cells in motor neuron disease, SOD1(G93A) transgenic mice were treated intracerebroventicularly (i.c.v.) with the anti-mitotic drug cytosine arabinoside (Ara-C).I.c.v. delivery of Ara-C accelerated disease progression in SOD1(G93A) mouse model of ALS.
|
22523565 |
2012 |
|
Motor Neuron Disease
|
|
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 Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
We next applied CME repeatedly to living Wld(S) mice and to SOD1(G93A) mice, an animal model of motor neuron disease, and observed degeneration of identified neuromuscular synapses over a 1-4day period in both of these mutant lines.
|
19683573 |
2009 |
|
Motor Neuron Disease
|
|
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 Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
Here we determined that dysregulated redox stress in ALS mice caused by NADPH oxidases Nox1 and Nox2 significantly influenced the progression of motor neuron disease caused by mutant SOD1(G93A) expression.
|
17853944 |
2007 |
|
Motor Neuron Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
Mice that overexpress the human Cu,Zn superoxide dismutase-1 mutant G93A develop a delayed and progressive motor neuron disease similar to human amyotrophic lateral sclerosis (ALS).
|
16049935 |
2005 |
|
Motor Neuron Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
To determine where and when the pathological changes of motor neuron disease begins, we performed a comprehensive spatiotemporal analysis of disease progression in SOD1(G93A) mice.
|
14736504 |
2004 |
|
Motor Neuron Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
Transgenic overexpression of Cu(+2)/Zn(+2) superoxide dismutase 1 (SOD1) harboring an amyotrophic lateral sclerosis (ALS)-linked familial genetic mutation (SOD1(G93A)) in a Sprague-Dawley rat results in ALS-like motor neuron disease.
|
11818550 |
2002 |
|
Motor Neuron Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
These findings suggest that the expression of the SOD1(G93A G1H) mutant protein results in a disease that resembles the late stages of human motor neuron disease.
|
12384220 |
2002 |
|
Motor Neuron Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
Nevertheless, four of the 22 G93A/p53-/- mice succumbed to MND after 160+/-28 days, as expected under these conditions of competing death risks if the absence of p53 fails to protect from MND.
|
11011020 |
2000 |
|
Motor Neuron Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
In the present study, we investigated the time course of microglial (major histocompatibility-II antigen immunoreactivity) and astrocytic (glial fibrillary acidic protein immunoreactivity) activation in relation to the course of motor neuron disease in the TgN(SOD1-G93A)G1H FALS mice.
|
9633809 |
1998 |
|
Motor Neuron Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
The onset of MND was delayed in these mice compared to the original G93A mice, but they developed the same neuropathologic abnormalities seen in the original G93A mice, albeit at a later time point with fewer vacuoles and more NF inclusions.
|
9382875 |
1997 |
|
Motor Neuron Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
Transgenic mice (line G1H) expressing a human SOD1 containing a mutation of Gly-93 --> Ala (G93A) develop a motor neuron disease similar to familial amyotrophic lateral sclerosis, but transgenic mice (line N1029) expressing a wild-type human SOD1 transgene do not.
|
8610185 |
1996 |
|
Motor Neuron Disease
|
|
0.100 |
GeneticVariation
|
BEFREE |
Expression of high levels of human SOD containing a substitution of glycine to alanine at position 93--a change that has little effect on enzyme activity--caused motor neuron disease in transgenic mice.
|
8209258 |
1994 |