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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
Mitochondrial pathology is a seminal pathogenic hallmark of familial amyotrophic lateral sclerosis (FALS) which is extensively manifested by human patients and mutant SOD1(G93A) mammalian models.
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27163198 |
2016 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
Using 1H-15N HSQC NMR spectroscopy, we have analyzed hydrogen exchange at the amide groups of wild-type (wt) CuZnSOD and the fALS-associated G93A SOD variant in their fully metalated states.
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19655787 |
2009 |
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Amyotrophic Lateral Sclerosis, Familial
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|
0.100 |
GeneticVariation
|
BEFREE |
Lentivirus and adeno-associated virus have been used to knockdown levels of mutated superoxide dismutase 1 (SOD1) in the G93A SOD1 mouse model of familial amyotrophic lateral sclerosis (fALS) to result in beneficial therapeutic outcomes.
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19495690 |
2009 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
In the present study, we injected MSCs into the cerebrospinal fluid of symptomatic hSOD1(G93A) rats, a transgenic animal model of familial amyotrophic lateral sclerosis (ALS) expressing a mutated form of the human superoxide dismutase.
|
19267424 |
2009 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
We examined the effect of rHuEPO in transgenic mice expressing the human Cu/Zn dependent-superoxide dismutase with G93A mutation (SOD1G93A), a model of familial amyotrophic lateral sclerosis. rHuEPO (4 unit/g BW s.c. three times/week), increased the haematocrit and induced a slight delay in impairment of motor function as measured by the rotating bar test.
|
17364433 |
2007 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
Recent studies suggest that microglia over-expressing mutant human superoxide dismutase (mSOD1(G93A)) may contribute to motoneuron death in a transgenic mouse model of familial amyotrophic lateral sclerosis.
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17555556 |
2007 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
The paper by Butterfield and colleagues reporting the use of redox proteomics to identify oxidatively modified proteins in the spinal cord in the G93A-SOD1 mouse model of familial amyotrophic lateral sclerosis was identified by the SCOPUS science literature information system to be one of the top 20 downloaded papers for 2005-2006 in Free Radical Biology and Medicine.
|
17603925 |
2007 |
|
Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
Human neuroblastoma SH-SY5Y cells transfected with either familial amyotrophic lateral sclerosis-typical G93A mutant or wild-type copper/zinc superoxide dismutase were compared to untransfected cells in term of glutamate transport.
|
15670639 |
2005 |
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Amyotrophic Lateral Sclerosis, Familial
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|
0.100 |
GeneticVariation
|
BEFREE |
Point mutations such as G93A and A4V in the human Cu/Zn-superoxide dismutase gene (hSOD1) cause familial amyotrophic lateral sclerosis (fALS).
|
16045483 |
2005 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
In this study, we demonstrate the actual role of a functional cross-talk between glial and neuronal cells expressing fALS mutant G93A-SOD1, where an increase in the production of reactive oxygen species occurs.
|
15208263 |
2004 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
Biophysical and biochemical analysis showed that zinc incorporation is severely reduced in the CuZnSOD proteins associated with the most severely forms of fALS (A4V, G93A).
|
15171736 |
2004 |
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Amyotrophic Lateral Sclerosis, Familial
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|
0.100 |
GeneticVariation
|
BEFREE |
To clarify the biological significance of the interaction of the redox system (Prx2/GPx1) with SOD1 in SOD1-mutated motor neurons in vivo, we produced an affinity-purified rabbit antibody against Prx2 and investigated the immunohistochemical localization of Prx2 and GPx1 in neuronal Lewy body-like hyaline inclusions (LBHIs) in the spinal cords of familial amyotrophic lateral sclerosis (FALS) patients with a two-base pair deletion at codon 126 and an Ala-->Val substitution at codon 4 in the SOD1 gene, as well as in transgenic rats expressing human SOD1 with H46R and G93A mutations.
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14648077 |
2004 |
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Amyotrophic Lateral Sclerosis, Familial
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|
0.100 |
GeneticVariation
|
BEFREE |
We examined its neuroprotective effect alone and in combination with the putative glutamate release blocker riluzole in the G93A model of familial amyotrophic lateral sclerosis (fALS).
|
15372249 |
2004 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
Dynamic properties of the G93A mutant of copper-zinc superoxide dismutase as detected by NMR spectroscopy: implications for the pathology of familial amyotrophic lateral sclerosis.
|
12590575 |
2003 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
Behavioural and anatomical effects of systemically administered leukemia inhibitory factor in the SOD1(G93A G1H) mouse model of familial amyotrophic lateral sclerosis.
|
12915243 |
2003 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
Degeneration of corticospinal and bulbospinal systems in the superoxide dismutase 1(G93A G1H) transgenic mouse model of familial amyotrophic lateral sclerosis.
|
12384220 |
2002 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
The aim of this study is to determine the effects of hNT Neuron transplants on motor neuron function in a mouse model of familial amyotrophic lateral sclerosis (FALS) in which there is a substitution of Alanine for Glycine at position 93 of the human SOD1 gene (G93A).
|
11922659 |
2002 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
In transgenic mice carrying the G93A human mutation of Cu/Zn superoxide dismutase (SOD1), which provide a model of familial amyotrophic lateral sclerosis, we investigated, before the onset of symptoms, two parameters of the response of facial motoneurons to nerve transection, i.e. nitric oxide synthase induction and motoneuron loss.
|
12421599 |
2002 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
In this study we investigated whether intense systemic LIF therapy prevents the loss of lumbar motoneurons in the transgenic SOD1 G93A mouse model of familial amyotrophic lateral sclerosis.
|
11730713 |
2001 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
In a recent work, we have observed that calcineurin activity is depressed in two models for familial amyotrophic lateral sclerosis (FALS) associated with mutations of the antioxidant enzyme Cu,Zn superoxide dismutase (SOD1), namely in neuroblastoma cells expressing either SOD1 mutant G93A or mutant H46R and in brain areas from G93A transgenic mice.
|
11701756 |
2001 |
|
Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
Transgenic mice carrying familial amyotrophic lateral sclerosis (FALS)-linked mutant Cu/Zn superoxide dismutase (SOD1) genes such as G93A (G93A-mice) and G85R (G85R-mice) genes develop limb paresis.
|
11860498 |
2001 |
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Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
Wild type CuZnSOD and several of the mutants associated with familial amyotrophic lateral sclerosis (FALS) (Ala(4) --> Val, Gly(93) --> Ala, and Leu(38) --> Val) were expressed in Saccharomyces cerevisiae.
|
10625639 |
2000 |
|
Amyotrophic Lateral Sclerosis, Familial
|
|
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, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
We have studied the effect of lysine acetylsalicylate (LAS; Aspegic), a soluble salt of aspirin, on motor deficits in transgenic mice expressing a human superoxide dismutase SOD1 mutation (Gly-93 --> Ala), an animal model of familial amyotrophic lateral sclerosis (FALS).
|
10072299 |
1999 |
|
Amyotrophic Lateral Sclerosis, Familial
|
|
0.100 |
GeneticVariation
|
BEFREE |
Three familial amyotrophic lateral sclerosis (FALS)-associated mutant Cu,ZnSODs, i.e., E100G, G93A, and G93R, did not differ from the control enzyme in susceptibility to inactivation by H2O2.
|
9587411 |
1998 |