
Pathological features of glial cells and motor neurons in the …
2023年1月15日 · In this study, we investigated whether similar degeneration is observed in AR2 mice. We compared the number of astrocytes and MNs in the lateral and medial AHs of the …
The AMPA receptor antagonist perampanel robustly rescues
2016年6月28日 · AR2 mice, in which an RNA editing enzyme adenosine deaminase acting on RNA 2 (ADAR2) is conditionally knocked out in the motor neurons, exhibit a progressive ALS …
Rescue of amyotrophic lateral sclerosis phenotype in a mouse …
Homozygous AR2 mice (n = 16) were injected with AAV9-hADAR2 vectors (2.14 × 10 12 vg/body) before (n = 11) or after (n = 5) the initiation of motor dysfunction that was defined by a decline …
When Does ALS Start? ADAR2–GluA2 Hypothesis for the Etiology …
2011年11月2日 · To investigate whether deficient ADAR2 activity plays pathogenic roles in sporadic ALS, we generated genetically modified mice (AR2) in which the ADAR2 gene was …
Rescue of amyotrophic lateral sclerosis phenotype in a mouse …
A single intravenous injection of AAV9-ADAR2 in conditional ADAR2 knockout mice (AR2), which comprise a mechanistic mouse model of sporadic ALS, caused expression of exogenous …
Negative features of sporadic amyotrophic lateral sclerosis: Motor ...
2024年8月15日 · Although facial and hypoglossal nerves were impaired in AR2 mice, cell death did not occur within the oculomotor nerve nucleus, as observed in patients with sporadic ALS. …
Rescue of amyotrophic lateral sclerosis phenotype in a mouse …
2013年9月24日 · A single intravenous injection of AAV9‐ADAR2 in conditional ADAR2 knockout mice (AR2), which comprise a mechanistic mouse model of sporadic ALS, caused expression …
Altered Intracellular Milieu of ADAR2-Deficient Motor Neurons in ...
2017年2月8日 · We discuss the contributors to ADAR2 downregulation and TDP-43 pathology in AR2 mouse motor neurons. We describe mechanisms of exaggerated Ca 2+ influx …
Pathological features of glial cells and motor neurons in the …
2023年1月15日 · In genetically modified mice (ADAR2 flox/flox /VAChT-Cre.Fast; AR2), the selective knockout of ADAR2 in cholinergic neurons induced progressive loss of lower MNs. …
Cell death cascade and molecular therapy in ADAR2-deficient …
Conditional ADAR2 knockout (AR2) mice display ALS-like phenotype, including progressive motor dysfunction due to loss of motor neurons. Motor neurons devoid of ADAR2 express Q/R site …
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