|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Thus, the ALS symptoms observed in G93A transgenic mice are not caused by the reduction of Cu,Zn-SOD activity with the mutant enzyme; rather, it is induced by a gain-of-function, an enhancement of the free radical-generating function.
|
8650157 |
1996 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
High levels of familial Amyotrophic Lateral Sclerosis (ALS)-linked SOD1 mutants G93A and G37R were previously shown to mediate disease in mice through an acquired toxic property.
|
9052802 |
1997 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Mice engineered to express a transgene encoding a human Cu/Zn superoxide dismutase (SOD1) with a Gly93 --> Ala (G93A) mutation found in patients who succumb to familial amyotrophic lateral sclerosis (FALS) develop a rapidly progressive and fatal motor neuron disease (MND) similar to amyotrophic lateral sclerosis (ALS).
|
9382875 |
1997 |
|
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 |
Three ALS mice [(SOD1-G93A)1Gur] and three controls [(SOD-1)2Gur] were obtained from The Jackson Laboratory.
|
9527889 |
1998 |
|
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 |
We used in vivo microdialysis to measure the conversion of 4-hydroxybenzoic acid to 3,4-dihydroxybenzoic acid (3,4-DHBA) as a measure of "hydroxyl radical-like" production in transgenic amyotrophic lateral sclerosis (ALS) mice with the G93A mutation as well as littermate controls.
|
9721759 |
1998 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Transgenic mice that express a mutant Cu,Zn-SOD, Gly93 --> Ala (G93A), have been shown to develop amyotrophic lateral sclerosis (ALS) symptoms.
|
10102580 |
1999 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
The fragmentation of the GA of Betz cells was identical to that previously reported in spinal cord motor neurons from patients with sporadic ALS and in transgenic mice expressing the G93A mutation of the gene encoding the Cu/Zn superoxide dismutase.
|
10223416 |
1999 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Consistent with this, we have demonstrated that overexpression of Bcl-2, a protein that inhibits apoptosis, attenuates neurodegeneration produced by the familial ALS-linked SOD1 mutant G93A (mSOD1).
|
10582606 |
1999 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Transgenic mice with several copies of a mutated human superoxide dismutase 1 (Gly93-Ala substitution) gene, i.e. a mutation responsible for the development of familial amyotrophic lateral sclerosis (ALS), integrated into the mouse genome, develop a slowly progressive paralysis of the hind-limbs accompanied by a corresponding degeneration of spinal cord neuronal tissue.
|
10726972 |
2000 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Transgenic mice expressing a mutated (G93A) human Cu/Zn superoxide dismutase (SOD1) develop motor neuron pathology and clinical symptoms similar to those seen in patients with amyotrophic lateral sclerosis.
|
10737625 |
2000 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Caspases play an instrumental role in neurodegeneration in transgenic mSOD1(G93A) mice, which suggests that caspase inhibition may have a protective role in ALS.
|
10764647 |
2000 |
|
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 |
To assess the role of p53-mediated apoptosis in amyotrophic lateral sclerosis, mice deficient in both p53 alleles (p53-/-) were crossed with transgenic mice expressing the G93A mutant (G93A+), creating novel transgenic knockout mice.
|
10964497 |
2000 |
|
Amyotrophic Lateral Sclerosis
|
|
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 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
These results show that a loss of GLT-1 protein is not necessary for ALS-like neurodegeneration in G93A mice.
|
11790392 |
2002 |
|
Amyotrophic Lateral Sclerosis
|
|
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 |
|
Amyotrophic Lateral Sclerosis
|
|
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.
|
12124437 |
2002 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Therefore, we undertook to look for folding-related defects by comparing the unfolding behavior of five ALS-associated mutants with distinct structural characteristics: A4V at the interface between the N and C termini, C6F in the hydrophobic core, D90A at the protein surface, and G93A and G93C, which decrease backbone flexibility.
|
12482932 |
2002 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
Its loss was investigated in the G93A mouse model of amyotrophic lateral sclerosis (ALS) in which the mutation of Cu/Zn superoxide dismutase (SOD1) is thought to lead to aberrant oxidative damage.
|
12514200 |
2003 |
|
Amyotrophic Lateral Sclerosis
|
|
0.100 |
GeneticVariation
|
BEFREE |
The backbone assignment of the copper-zinc superoxide dismutase amyotrophic lateral sclerosis G93A mutant was performed on an (15)N-enriched protein sample.
|
12590575 |
2003 |
|
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 |
In mice that express the mutant human gene Cu/Zn superoxide dismutase G93A (SOD1), and therefore simulate the chronic human motor neuron disease amyotrophic lateral sclerosis, Cop-1 vaccination prolonged life span compared to untreated matched controls, from 211 +/- 7 days (n = 15) to 263 +/- 8 days (n = 14; P < 0.0001).
|
12668759 |
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 |