|
Amyotrophic Lateral Sclerosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Furthermore, TDP-43 pathology, the pathological hallmark of ALS, is observed in the ADAR2-lacking motor neurons in ALS patients and conditional ADAR2 knockout mice, suggesting a pivotal role of ADAR2 downregulation in the ALS pathogenesis.
|
30448461 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our findings suggest that the mislocalization of ADAR2 in C9orf72 mediated ALS/FTD is responsible for the alteration of RNA processing events that may impact vast cellular functions, including the integrated stress response (ISR) and protein translation.
|
30945056 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
These lines of evidence prompted us to develop molecular targeting therapy for ALS by normalization of disrupted intracellular environment due to ADAR2 down-regulation.
|
29944911 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Therefore, normalization of disrupted intracellular environments resulting from ADAR2 downregulation may be a therapeutic target for ALS.
|
28208729 |
2017 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Analogous changes were observed in motor neurons lacking ADAR2 immunoreactivity in sporadic ALS patients.
|
28045133 |
2017 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
ADAR2 deficiency can occur in ALS patients with a FUS(P525L) mutation and is unrelated to the presence of FUS-positive inclusions.
|
27343041 |
2016 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Elucidation of the molecular cascade of neuronal death induced by ADAR2 downregulation could provide a new specific therapy for sporadic ALS.
|
24355598 |
2014 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
This AAV9-mediated ADAR2 gene delivery may therefore enable the development of a gene therapy for ALS.
|
24115583 |
2013 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
In addition, we analyzed the enzymatic activity of three members of the ADAR family (ADAR1, ADAR2 and ADAR3) in ALS motor neurons expressing unedited GluA2 mRNA and those expressing only edited GluA2 mRNA.
|
22226999 |
2012 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
We found that all motor neurons were ADAR2-positive in the control cases, whereas more than half of them were ADAR2-negative in the ALS cases.
|
20372915 |
2010 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our review will include a discussion of new ADAR2 substrates that may be useful for research on sporadic ALS.
|
20424386 |
2010 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Therefore, the extent of CYFIP2 K/E site-editing may be an additional marker for ADAR2 activity in neuronal and other types of cells in vivo, as well as in vitro, and thus is considered to be a good tool for sporadic ALS research.
|
18971634 |
2009 |
|
Amyotrophic Lateral Sclerosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Because ADAR2 underactivity may be a causative molecular change of death of motor neurons in sporadic amyotrophic lateral sclerosis (ALS), this newly identified ADAR2-mediated editing position may become a useful tool for ALS research.
|
18407364 |
2008 |