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�T 英文原著

　　　　　
1. Aizawa H, Yamashita T, Kato H, Kimura T, Kwak S.: Impaired Nucleoporins Are Present in Sporadic Amyotrophic Lateral Sclerosis Motor Neurons that Exhibit Mislocalization of the 43-kDa TAR DNA-Binding Protein. J Clin Neurol.2019.1. 15(1):62-67.doi:10.3988/jcn.2019.15.1.62
　　　　　
2. Takahira Shirahase,Yoshihisa Watanabe,Atsushi Tsujimura,Shin Kwak,Toshiro Yamamoto,Narisato Kanamura and Masaki Tanaka: Ethanol Preference and Drinking Behavior Are Controlled by RNA Editing in the Nucleus Accumbens. Front.Behav.Neurosci. 2019.1.15.doi:10.1.10.3389/fnbeh.2018.00331
　　　　　
3. Hosaka T, Yamashita T, Teramoto S, Hirose N, Tamaoka A, Kwak S.: ADAR2-dependent A-to-I RNA editing in the extracellular linear and circular RNAs. Neurosci Res 2018.11.16. pii: S0168-0102(18)30587-X.
   doi: 10.1016/j.neures.2018.11.005.
　　　　　
4. Yamashita T, Kwak S:Cell death cascade and molecular therapy in ADAR2-deficient motor neurons of ALS.Neurosci Res 2019 Jul;144:4-13.
   doi:10.1016/j.neures.2018.06.004.
　　　　　
5. Takenari Yamashita, Hitoshi Aizawa, Sayaka Teramoto, Megumi Akamatsu & Shin Kwak: Calpain-dependent disruption of nucleo-cytoplasmic transport in ALS motor neurons. Scientific Reports 7:39994,2017.1.3.
   doi: 10.1038/srep39994.
　　　　　
6. Akamatsu M, Yamashita T, Hirose N, Teramoto S, Kwak S: The AMPA receptor antagonist perampanel robustly rescues amyotrophic lateral sclerosis (ALS) pathology in sporadic ALS model mice. Scientific Reports 7:28649,2016.6.28.doi:10.1038/srep28649.
　　　　　
7. Aizawa H, Hideyama T, Yamashita T, Kimura T, Suzuki N, Aoki M, Kwak S: Deficient RNA-editing enzyme ADAR2 in an amyotrophic lateral sclerosis patient with a FUSP525L mutation. Journal of Clinical Neuroscience 32: 128-129, 2016.6.21.doi:10.1016/j.jocn.2015.12.039.
　　　　　
8. Yamashita T, Teramoto S, Kwak S: Phosphorylated TDP-43 becomes resistant to cleavage by calpain: a regulatory role for phosphorylation in TDP-43 pathology of ALS/FTLD. Neuroscience Research 107:63-69,2016.6.1. doi:10.1016/j.neures.2015.12.006.
　
9. Sasaki S, Yamashita T, Kwak S: Autophagy in spinal motor neurons of conditional ADAR2-knockout mice: an implication for a role of calcium in increased autophagy flux in ALS. Neuroscience Letters 598:79-84,2015. doi:10.1016/j.neulet.2015.05.025.(Corrigendum 602:173,2015)
　
10. Yamashita T, Kwak S：The molecular link between inefficient GluA2 Q/R site-RNA editing and TDP-43 pathology in motor neurons of sporadic amyotrophic lateral sclerosis patients. Journal of Brain Research 1585:28-38,2014/10.10. doi.org/10.1016/j.brainres.2013.12.011.


11. Sasaki S, Yamashita T, Hideyama T, Kwak S: Unique nuclear vacuoles in the motor neurons of conditional ADAR2-knockout mice. Journal of Brain Research 1550:536-546,2014.3.6.doi: 10.1016/j.brainres.2014.01.006.


12. Akamatsu M, Takuma H, Yamashita T, Okada T, Keino-Masu K, Ishii K, Kwak S, Masu M, Tamaoka A: A unique mouse model for investigating the properties of amyotrophic lateral sclerosis-associated protein TDP-43, by in utero electroporation. Neuroscience Research 77(4):234-41,2013. doi:10.1016/j.neures.2013.09.009


13. Yamashita T, Chai HL, Teramoto S, Tsuji S, Shimazaki K, Muramatsu S, Kwak S: Rescue of amyotrophic lateral sclerosis phenotype in a mouse model by intravenous AAV9-ADAR2 delivery to motor neurons. EMBO Molecular Medicine 5(11):171,2013.2013.9.24.doi:10.1002/emmm.201302935.


14. Al-Chalabi A, Kwak S, Mehler M, Rouleau G, Siddique T, Strong M, Leigh PN: Genetic and epigenetic studies of amhyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 14 (Supple 1) 44-52, 2013.
    doi: 10.3109/21678421.2013.778571.


15. Shin Kwak: Molecular Link between Inefficient GluA2 RNA Editing and TDP-43 Pathology in ALS Motor Neurons. Brain and Nerve 64:5,549-556,2012


16. Yamashita T, Hideyama T, Hachiga K, Teramoto S, Takano J, Iwata N, Saido TC, and  Kwak S: A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology. Nature Communications 3:1307,2012. doi:10.1038/ncomms2303,2012


17. Hideyama T, Teramoto S, Hachiga K, Yamashita T, Kwak S: Co-occurrence of TDP-43 mislocalization with reduced RNA editing enzyme, ADAR2, in aged mouse motor neurons: implications for age-related acceleration of ALS. PLoSOne 7(8):e43469, 2012.10.1371/journal.pone.0043469 .


18. Yamashita T, Hideyama T, Teramoto S, Kwak S: Abnormal processing of TDP-43 does not regulate ADAR2 activity in cultured cell lines. Neuroscience Research 73: 153-160, 2012.doi:10.1016/j.neures.2012.02.015.


19. Yamashita T, Tadami C, Nishimoto Y, Hideyama T, Kimura D, Suzuki T, Kwak S: RNA editing of the Q/R site of GluA2 in different cultured cell lines that constitutively express different levels of RNA editing enzyme ADAR2. Neuroscience Research 73: 42-48, 2012.doi:10.1016/j.neures.2012.02.002.


20. Hideyama T, Yamashita T, Aizawa H, Tsuji S, Kakita A, Takahashi H, Kwak S: Profound downregulation of the RNA editing enzyme ADAR2 in ALS spinal motor neurons. Neurobiology of Disease 45:1121-28, 2012. doi:10.1016/j.nbd.2011.12.033


21. Hideyama T, Kwak S: When does ALS start? ADAR2-GluA2 hypothesis for the etiology of sporadic ALS. Frontier MolecularNeuroscience 4:33, 2011. doi:10.3389/fnmol.2011.00033


22. Kwak S, Hideyama T, Yamashita T, Aizawa H: AMPA receptor-mediated neuronal death in sporadic ALS. Neuropathology 30:182-188,2010,


23. Hideyama T, Yamashita T, Suzuki T, Tsuji S, Higuchi M, Seeburg PH, Takahashi R, Misawa H, Kwak S: Induced loss of ADAR2 engenders slow death of motor neurons from Q/R site-unedited GluR2. The Journal of Neuroscience 30: 11917-11925, 2010. doi:10.1523/JNEUROSCI.2021-10.2010.


24. Aizawa H, Sawada J, Hideyama T, Yamashita T, Katayama T, Hasebe N, KimuraT, Yahara O, Kwak S: TDP-43 pathology in sporadic ALS occurs in motor neurons lacking RNA editing enzyme ADAR2. Acta Neuropathologica 120: 75-84, 2010.doi:10.1007/s00401-010-0678-x.


25. Sawada J, Yamashita T (contributed equally with JS), Aizawa H, Aburakawa Y, Hasebe N, Kwak S: Effects of antidepressants on GluR2 Q/R site-RNA editing in a modified HeLa cell line. Neuroscience Research 64:251-258, 2009. doi:10.1016/j.neures.2009.03.009.


26. Nishimoto Y, Yamashita T (contributed equally with YN), Hideyama T, Tsuji S, Suzuki N, Kwak S: Determination of editors of mRNAs with site-selective A-to-I editing positions. Neuroscience Research 61: 201-206, 2008. doi:10.1016/j.neures.2008.02.009


27. Iwata NK, Aoki S, Okabe S, Arai N, Terao Y, Kwak S, Abe O, Kanazawa I, Tsuji S, Ugawa Y: Evaluation of corticospinal tracts in ALS with diffusion tensor MRI and brainstem stimulation. Neurology 70: 528-32, 2008. doi:10.1212/01.wnl.0000299186.72374.19


28. Hideyama T, Momose T, Shimizu J, Tsuji S, Kwak S: A PET study on the role of nigral lesions in parkinsonism in patients with ALS. Arch Neurol 63:1719-1722, 2006.doi:10.1001/archneur.63.12.1719


29. Sun H, Kawahara Y, Ito K, Kanazawa I, Kwak S: Slow and selective death of spinal motor neurons in vivo by intrathecal infusion of kainic acid: implications for AMPA receptor-mediated excitotoxicity in ALS. Journal of Neurochemistry 98:782-791, 2006.doi:0.1111/j.1471-4159.2006.03903.x


30. Kawahara Y, Sun H, Ito K, Hideyama T, Aoki M, Sobue G, Tsuji S, Kwak S: Underediting of GluR2 mRNA, a neuronal death inducing molecular change in sporadic ALS, does not occur in motor neurons in SBMA patients or SOD1 transgenic rats. Neuroscience Research 54:11-15, 2006. doi:10.1016/j.neures.2005.09.006.


31. Kawahara Y, Ito K, Ito M, Tsuji S, Kwak S: Novel splice variants of human ADAR2 mRNA: skipping of the exon encoding the dsRNA-binding domains, and multiple C-terminal splice sites. Gene 363: 193-201, 2005. doi:10.1016/j.gene.2005.07.028


32. Yamamoto Y, Struzik ZR, Soma R, Ohashi K, Kwak S: Noisy vestibular stimulation improves autonomic and motor responsiveness in central neuro-degenerative disorders. Annals of Neurology 58: 175-181, 2005. doi:10.1002/ana.20574


33. Aoki S, Iwata NK, Masutani Y, Yoshida M, Abe O, Ugawa Y, Masumoto T, Mori H, Hayashi N, Kabasawa H, Kwak S, Takahashi S, Tsuji S, Ohtomo K: Quantitative evaluation of the pyramidal tract segmented by diffusion tensor tractography: feasibility study in patients with amyotrophic lateral sclerosis. Radiation Medicine 23: 195-199, 2005. https://www.ncbi.nlm.nih.gov/pubmed/15940067


34. Sun H, Kawahara Y, Ito K, Kanazawa I, Kwak S: Expression profile of AMPA receptor subunit mRNA in single adult rat brain and spinal cord neurons in situ. Neuroscience Research 52:228-234, 2005.doi:10.1016/j.neures.2005.03.008.


35. Kawahara Y, Ito K, Sun H, Ito M, Kanazawa I, Kwak S: GluR4c, an alternative splicing isoform of GluR4 is abundantly expressed in adult human brain. Molecular Journal of Brain Research 127：150-155, 2004.doi:10.1016/j.molbrainres.2004.05.020.


36. Kawahara Y, Ito K, Sun H, Aizawa H, Kanazawa I, Kwak S: RNA editing and death of motor neurons. Nature 427:801, 2004.doi:10.1038/427801a.


37. Kawahara Y, Ito K, Sun H, Ito M, Kanazawa I, Kwak S: Regulation of glutamate receptor RNA editing and ADAR mRNA expression in developing human normal and Down’s syndrome brains. Developmental Journal of Brain Research 148：151-155, 2004. doi:10.1016/j.devbrainres.2003.11.008.


38. Kawahara Y, Ito K, Sun H, Kanazawa I, Kwak S: Low editing efficiency of GluR2 mRNA is associated with a low relative abundance of ADAR2 mRNA in white matter of normal human brain. European Journal of Neuroscience 18:23-33, 2003. doi:10.1046/j.1460-9568.2003.02718.x.


39. Kawahara Y, Kwak S, Sun H, Ito K, Hashida H, Aizawa H, Jeong S-Y, Kanazawa I: Human spinal motoneurons express low relative abundance of GluR2 mRNA: An implication for excitotoxicity in ALS. Jornal of Neurochemistry 85:680-689, 2003.doi:10.1046/j.1471-4159.2003.01703.x.


40. Takuma H, Kwak S, Yoshizawa T, Kanazawa I: Reduction of GluR2 RNA editing, a molecular change that increases calcium influx through AMPA receptors, selective in the spinal ventral gray of patients with amyotrophic lateral sclerosis. Annals of Neurology 46:806-815, 1999.