|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Generation of an induced pluripotent stem cell line, ICGi014-A, by reprogramming peripheral blood mononuclear cells from a patient with homozygous D90A mutation in SOD1 causing Amyotrophic lateral sclerosis.
|
31830646 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
In addition, those pathological neurofilament accumulations are known in α-synuclein in Parkinson's disease (PD), Aβ and tau in Alzheimer's disease (AD), polyglutamine in CAG trinucleotide repeat disorders, superoxide dismutase 1 (SOD1), TAR DNA-binding protein 43 (TDP43), neuronal FUS proteins, optineurin (OPTN), ubiquilin 2 (UBQLN2), and dipeptide repeat protein (DRP) in amyotrophic lateral sclerosis (ALS).
|
31820696 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
To inhibit the abnormal aggregation of Cu, Zn-superoxide dismutase (SOD1) is regarded as a potential therapeutic strategy of SOD1-linked amyotrophic lateral sclerosis (ALS).
|
31820923 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
The ROC analysis demonstrated that the ECAS-ALS nonspecific score (comprising memory and visuospatial domains) is the most sensitive and specific in differentiating the AD from ALS patients.
|
31469297 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
Recently, Edaravone has been licensed in several countries for the treatment of ALS based on a randomized controlled trial in a selected group of ALS patients excluding the EEC category "clinically probable laboratory supported".
|
31561715 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
The mutations in Cu/Zn superoxide dismutase (SOD1) causing its misfolding and aggregation are found linked to the motor neuron disorder, amyotrophic lateral sclerosis.
|
31669277 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Compensatory changes in degenerating spinal motoneurons sustain functional sparing in the SOD1-G93A mouse model of amyotrophic lateral sclerosis.
|
31364764 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
Mutants of Cu,Zn-superoxide dismutase (SOD1) exhibit cytotoxicity such as aggregation and pro-oxidation after denaturation, which is thought to be involved in the pathogenesis of amyotrophic lateral sclerosis (ALS).
|
31348988 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
Thus, we tested male and female SOD1 <sup>G93A</sup> mice for the first time before the onset of debilitating motor impairments in behavioural domains relevant to both ALS and frontotemporal dementia.
|
31412164 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
C9orf72, SOD1, TARDBP, and FUS are noted as the most common ALS genes; however, mutations of these genes explain <10% of sALS cases.
|
31060816 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
Using a dietary supplementation, we increased DHA levels (2% mean increase, p < 0.01) in the LSC of the familial ALS murine model B6SJL-Tg(SOD1*G93A)1Gur/J.
|
31755041 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
A 20% of familial ALS cases are associated with mutations in the gene coding for superoxide dismutase 1 (SOD1).
|
31176720 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
The effects were greatest in cells derived from patients carrying ALS-linked mutations in SOD1.
|
30863976 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Overview of Impaired BDNF Signaling, Their Coupled Downstream Serine-Threonine Kinases and SNARE/SM Complex in the Neuromuscular Junction of the Amyotrophic Lateral Sclerosis Model SOD1-G93A Mice.
|
30929165 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Histamine Is an Inducer of the Heat Shock Response in SOD1-G93A Models of ALS.
|
31382568 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Mutations in Cu-Zn superoxide dismutase 1 (SOD1) cause familial forms of amyotrophic lateral sclerosis (ALS).
|
30841754 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
Mutations in the gene encoding Cu/Zn Superoxide Dismutase 1 (SOD1) protein are contemplated to be a protruding reason for Amyotrophic lateral sclerosis (ALS), which leads towards protein aggregation, misfolding and destabilization.
|
30701485 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
The combined proteomic analysis allowed the detection of regulated proteins linked to ALS pathogenesis (RNA-binding protein FUS, superoxide dismutase Cu-Zn and neurofilaments light polypeptide) alongside newly identified candidate biomarkers (myosin-9, fructose-bisphosphate aldolase and plectin).
|
30872628 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
Further, reduced membralin and EAAT2 levels correlated with disease progression in spinal cord from SOD1-mutant mouse models, and reductions in membralin/EAAT2 were observed in human ALS spinal cord.
|
31112137 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
SOD1 activity threshold and TOR signalling modulate VAP(P58S) aggregation via reactive oxygen species-induced proteasomal degradation in a <i>Drosophila</i> model of amyotrophic lateral sclerosis.
|
30635270 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Most of reports describing ASL-FTD cases show a strong association between ALS and the behavioural form of FTD.
|
30146930 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
The actions of TRVA242 were also conserved in a mammalian model as it also stabilized neuromuscular junction deficits in a mouse SOD1 model of ALS.
|
31342410 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
GeneticVariation
|
disease |
BEFREE |
Data from clinical studies show that multiple genetic mutations linked to ALS (eg, mutations in SOD1, TARDBP, and C9orf72) enhance this neuroinflammation, which provides compelling evidence for immune dysregulation in the pathogenesis of ALS.
|
30663610 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
We propose that translational incorporation of BMAA into SOD1 is directly responsible for its toxicity in neurodegeneration, and BMAA modification of SOD1 may serve as a biomarker of ALS.
|
31323035 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.800 |
Biomarker
|
disease |
BEFREE |
In SOD1G93A/CX3CR1-/- mice demonstrated more neuronal cell loss, more microglial activation and exacerbated SOD1 aggregation at the end-stage of ALS.
|
30352020 |
2019 |