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lüll Novel etiological and therapeutic strategies for neurodiseases: RNA editing enzyme abnormality in sporadic amyotrophic lateral sclerosis Hideyama T; Yamashita T; Nishimoto Y; Suzuki T; Kwak SJ Pharmacol Sci 2010[]; 113 (1): 9-13The motor neurons of patients with sporadic amyotrophic lateral sclerosis (ALS) express abundant Q/R site-unedited GluR2 mRNA, whereas those of patients with other motor neuron diseases including familial ALS associated with mutated SOD1 (ALS1) and those of normal subjects express only Q/R site-edited GluR2 mRNA. Because adenosine deaminase acting on RNA type 2 (ADAR2) specifically catalyzes GluR2 Q/R site-editing, it is likely that ADAR2 activity is not sufficient to edit this site completely in motor neurons of patients with sporadic ALS. Because these molecular abnormalities occur in disease- and motor neuron-specific fashion and induce fatal epilepsy in mice, we have hypothesized that GluR2 Q/R site-underediting due to ADAR2 underactivity is a cause of neuronal death in sporadic ALS. We found that cytoplasmic fragile X mental retardation protein interacting protein 2 (CYFIP2) mRNA had an ADAR2-mediated editing position using RNA interference knockdown. Our review will include a discussion of new ADAR2 substrates that may be useful for research on sporadic ALS.|*RNA Editing[MESH]|Adaptor Proteins, Signal Transducing/*genetics[MESH]|Adenosine Deaminase/*genetics/metabolism[MESH]|Amyotrophic Lateral Sclerosis/enzymology/*genetics[MESH]|Animals[MESH]|Cell Death/genetics[MESH]|Humans[MESH]|Mice[MESH]|Mice, Knockout[MESH]|Models, Biological[MESH]|Motor Neurons/*metabolism[MESH]|RNA-Binding Proteins[MESH]|Receptors, AMPA/genetics[MESH] |