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- W2045245162 abstract "TDP-43 pathology in motor neurons is a hallmark of ALS. In addition, the reduced expression of an RNA editing enzyme, adenosine deaminase acting on RNA 2 (ADAR2), increases the expression of GluA2 with an unedited Q/R site in the motor neurons of patients with sporadic ALS. As the occurrence of these two disease-specific abnormalities in the same motor neurons suggests a molecular link between them, we examined the effects of altered TDP-43 processing on ADAR2 activity in TetHeLaG2m and Neuro2a cells. We found that ADAR2 activity did not consistently change due to the overexpression or knockdown of TDP-43 or the expression of abnormal TDP-43, including caspase-3-cleaved fragments, truncated TDP-43 lacking either nuclear localization or export signals and ALS-linked TDP-43 mutants. These results suggest that the abnormal processing of TDP-43 is not an upstream event of inefficient GluA2 Q/R site editing in the motor neurons of sporadic ALS patients." @default.
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- W2045245162 date "2012-06-01" @default.
- W2045245162 modified "2023-09-23" @default.
- W2045245162 title "The abnormal processing of TDP-43 is not an upstream event of reduced ADAR2 activity in ALS motor neurons" @default.
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- W2045245162 doi "https://doi.org/10.1016/j.neures.2012.02.015" @default.
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