Shin Kwak

Summary

Affiliation: University of Tokyo
Country: Japan

Publications

  1. ncbi request reprint Glutamate receptors: RNA editing and death of motor neurons
    Yukio Kawahara
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Nature 427:801. 2004
  2. doi request reprint AMPA receptor-mediated neuronal death in sporadic ALS
    Shin Kwak
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo
    Neuropathology 30:182-8. 2010
  3. ncbi request reprint Newly identified ADAR-mediated A-to-I editing positions as a tool for ALS research
    Shin Kwak
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Bunkyo ku, Tokyo, Japan
    RNA Biol 5:193-7. 2008
  4. ncbi request reprint Deficient RNA editing of GluR2 and neuronal death in amyotropic lateral sclerosis
    Shin Kwak
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, 113 8655 Tokyo, Japan
    J Mol Med (Berl) 83:110-20. 2005
  5. doi request reprint Induced loss of ADAR2 engenders slow death of motor neurons from Q/R site-unedited GluR2
    Takuto Hideyama
    Core Research for Evolutional Science and Technology, Japan Science and Technology Agency, University of Tokyo, Bunkyo ku, Tokyo, Japan
    J Neurosci 30:11917-25. 2010
  6. ncbi request reprint Underediting of GluR2 mRNA, a neuronal death inducing molecular change in sporadic ALS, does not occur in motor neurons in ALS1 or SBMA
    Yukio Kawahara
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Neurosci Res 54:11-4. 2006
  7. ncbi request reprint Novel etiological and therapeutic strategies for neurodiseases: RNA editing enzyme abnormality in sporadic amyotrophic lateral sclerosis
    Takuto Hideyama
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, Japan
    J Pharmacol Sci 113:9-13. 2010
  8. ncbi request reprint Slow and selective death of spinal motor neurons in vivo by intrathecal infusion of kainic acid: implications for AMPA receptor-mediated excitotoxicity in ALS
    Hui Sun
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
    J Neurochem 98:782-91. 2006
  9. doi request reprint RNA editing of the Q/R site of GluA2 in different cultured cell lines that constitutively express different levels of RNA editing enzyme ADAR2
    Takenari Yamashita
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Hongo, Bunkyo ku, Tokyo, Japan
    Neurosci Res 73:42-8. 2012
  10. ncbi request reprint Human spinal motoneurons express low relative abundance of GluR2 mRNA: an implication for excitotoxicity in ALS
    Yukio Kawahara
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, Hongo, Tokyo, Japan
    J Neurochem 85:680-9. 2003

Collaborators

Detail Information

Publications29

  1. ncbi request reprint Glutamate receptors: RNA editing and death of motor neurons
    Yukio Kawahara
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Nature 427:801. 2004
    ....
  2. doi request reprint AMPA receptor-mediated neuronal death in sporadic ALS
    Shin Kwak
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo
    Neuropathology 30:182-8. 2010
    ..To this end, normalization of ADAR2 activity in motor neurons may become a therapeutic strategy for sporadic ALS...
  3. ncbi request reprint Newly identified ADAR-mediated A-to-I editing positions as a tool for ALS research
    Shin Kwak
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Bunkyo ku, Tokyo, Japan
    RNA Biol 5:193-7. 2008
    ..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...
  4. ncbi request reprint Deficient RNA editing of GluR2 and neuronal death in amyotropic lateral sclerosis
    Shin Kwak
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, 113 8655 Tokyo, Japan
    J Mol Med (Berl) 83:110-20. 2005
    ..The restoration of this enzyme activity in ALS motor neurons may open the novel strategy for specific ALS therapy...
  5. doi request reprint Induced loss of ADAR2 engenders slow death of motor neurons from Q/R site-unedited GluR2
    Takuto Hideyama
    Core Research for Evolutional Science and Technology, Japan Science and Technology Agency, University of Tokyo, Bunkyo ku, Tokyo, Japan
    J Neurosci 30:11917-25. 2010
    ..2000). Thus, loss of ADAR2 activity causes AMPA receptor-mediated death of motor neurons...
  6. ncbi request reprint Underediting of GluR2 mRNA, a neuronal death inducing molecular change in sporadic ALS, does not occur in motor neurons in ALS1 or SBMA
    Yukio Kawahara
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Neurosci Res 54:11-4. 2006
    ..It seems likely that the death cascade is different among the dying motor neurons in sporadic ALS, familial ALS with mutant SOD1 and SBMA...
  7. ncbi request reprint Novel etiological and therapeutic strategies for neurodiseases: RNA editing enzyme abnormality in sporadic amyotrophic lateral sclerosis
    Takuto Hideyama
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, Japan
    J Pharmacol Sci 113:9-13. 2010
    ..Our review will include a discussion of new ADAR2 substrates that may be useful for research on sporadic ALS...
  8. ncbi request reprint Slow and selective death of spinal motor neurons in vivo by intrathecal infusion of kainic acid: implications for AMPA receptor-mediated excitotoxicity in ALS
    Hui Sun
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
    J Neurochem 98:782-91. 2006
    ..e. calcium-permeable) AMPA receptors. This rat model may be useful in investigating ALS etiology...
  9. doi request reprint RNA editing of the Q/R site of GluA2 in different cultured cell lines that constitutively express different levels of RNA editing enzyme ADAR2
    Takenari Yamashita
    Department of Neurology, Graduate School of Medicine, University of Tokyo, Hongo, Bunkyo ku, Tokyo, Japan
    Neurosci Res 73:42-8. 2012
    ....
  10. ncbi request reprint Human spinal motoneurons express low relative abundance of GluR2 mRNA: an implication for excitotoxicity in ALS
    Yukio Kawahara
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, Hongo, Tokyo, Japan
    J Neurochem 85:680-9. 2003
    ..However, the low relative abundance of GluR2 might provide spinal motoneurons with conditions that are easily affected by changes of AMPA receptor properties including deficient GluR2 mRNA editing in ALS...
  11. ncbi request reprint Low editing efficiency of GluR2 mRNA is associated with a low relative abundance of ADAR2 mRNA in white matter of normal human brain
    Yukio Kawahara
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Eur J Neurosci 18:23-33. 2003
    ..These results suggest that Q/R site of GluRs editing is regulated in a regional, and hence presumably cell-specific, manner and that the GluR2 Q/R site editing is critically regulated by ADAR2 in human brain...
  12. doi request reprint Profound downregulation of the RNA editing enzyme ADAR2 in ALS spinal motor neurons
    Takuto Hideyama
    CREST, Japan Science and Technology Agency, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Neurobiol Dis 45:1121-8. 2012
    ..These results indicate that ADAR2 downregulation is a profound pathological change relevant to death of motor neurons in ALS...
  13. doi request reprint Determination of editors at the novel A-to-I editing positions
    Yoshinori Nishimoto
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Neurosci Res 61:201-6. 2008
    ..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...
  14. ncbi request reprint Expression profile of AMPA receptor subunit mRNA in single adult rat brain and spinal cord neurons in situ
    Hui Sun
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Neurosci Res 52:228-34. 2005
    ..The unique AMPA receptor expression profile of motoneurons may render them selectively vulnerable to AMPA receptor-mediated excitotoxicity...
  15. ncbi request reprint [AMPA receptor-mediated neuronal death and amyotrophic lateral sclerosis]
    Takuto Hideyama
    Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    No To Shinkei 57:585-98. 2005
  16. ncbi request reprint GluR4c, an alternative splicing isoform of GluR4, is abundantly expressed in the adult human brain
    Yukio Kawahara
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo, Tokyo 113 8655, Japan
    Brain Res Mol Brain Res 127:150-5. 2004
    ..The GluR4 subunit may play a pivotal role in regulating channel properties as well as trafficking of alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionate (AMPA) receptors in the adult human brain...
  17. doi request reprint The abnormal processing of TDP-43 is not an upstream event of reduced ADAR2 activity in ALS motor neurons
    Takenari Yamashita
    CREST, Japan Science and Technology Agency, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Neurosci Res 73:153-60. 2012
    ..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...
  18. ncbi request reprint Novel splice variants of human ADAR2 mRNA: skipping of the exon encoding the dsRNA-binding domains, and multiple C-terminal splice sites
    Yukio Kawahara
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Gene 363:193-201. 2005
    ..Among them, variants containing the long C-terminus are translated in human brains in situ, implying that alternative splicing in the 3' UTR of ADAR2 might regulate translational efficiency and mRNA stability in vivo...
  19. ncbi request reprint Regulation of glutamate receptor RNA editing and ADAR mRNA expression in developing human normal and Down's syndrome brains
    Yukio Kawahara
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo, Tokyo 113 8655, Japan
    Brain Res Dev Brain Res 148:151-5. 2004
    ..ADAR2 mRNA was not overexpressed in the brains of Down's syndrome subjects, nor was there any evidence of changes in the RNA editing efficiency of their GluRs...
  20. doi request reprint A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology
    Takenari Yamashita
    CREST, Japan Science and Technology Agency, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 0033, Japan
    Nat Commun 3:1307. 2012
    ....
  21. ncbi request reprint Calcium-permeable AMPA channels in neurodegenerative disease and ischemia
    Shin Kwak
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, 113 8655 Tokyo, Japan
    Curr Opin Neurobiol 16:281-7. 2006
    ..The targeting of these channels might provide efficacious new avenues in the therapy of certain neurological diseases...
  22. ncbi request reprint Recent advances in the study of AMPA receptors
    Takeshi Suzuki
    Department of Pharmacology, Kyoritsu College of Pharmacy, Tokyo, Japan
    Nihon Yakurigaku Zasshi 122:515-26. 2003
    ..Glioblastoma cells also express Ca(2+)-permeable AMPA receptors, and their conversion to Ca(2+)-impermeable receptors inhibits cell locomotion and induces apoptosis...
  23. ncbi request reprint [Molecular link between inefficient GluA2 RNA editing and TDP-43 pathology in ALS motor neurons]
    Shin Kwak
    Department of Neurology, The University of Tokyo, Tokyo, Japan
    Brain Nerve 64:549-56. 2012
    ....
  24. ncbi request reprint A positron emission tomography study on the role of nigral lesions in parkinsonism in patients with amyotrophic lateral sclerosis
    Takuto Hideyama
    Department of Neurology, Graduate School of Medicine, and Department of Radiology, The University of Tokyo, Tokyo, Japan
    Arch Neurol 63:1719-22. 2006
    ..Patients with amyotrophic lateral sclerosis (ALS) sometimes exhibit parkinsonism, but the lesion responsible for parkinsonism has not been extensively studied...
  25. doi request reprint Age at onset influences on wide-ranged clinical features of sporadic amyotrophic lateral sclerosis
    Naoki Atsuta
    Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan
    J Neurol Sci 276:163-9. 2009
    ..To profile the detailed clinical features of sporadic amyotrophic lateral sclerosis (ALS) on large-scale samples in Japan...
  26. doi request reprint Aceruloplasminemia in a Japanese woman with a novel mutation of CP gene: clinical presentations and analysis of genetic and molecular pathogenesis
    Ayumi Hida
    Department of Neurology, Graduate School of Medicine, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    J Neurol Sci 298:136-9. 2010
    ..Thus, our report on this novel mutant indicates the previously unreported importance of carboxy-terminus residues in the secretion pathway...
  27. ncbi request reprint [ALS and excitatory amino acid]
    Hitoshi Aizawa
    Division of Neurology, Department of Internal Medicine, Asahik awa Medical College, 2 1 1 1 Midorigaoka Higashi, Asahikawa 078 8510, Japan
    Brain Nerve 59:1117-27. 2007
    ....
  28. ncbi request reprint Excitotoxicity and ALS: what is unique about the AMPA receptors expressed on spinal motor neurons?
    Yukio Kawahara
    The Wistar Institute, Philadelphia, PA 19104, USA
    Amyotroph Lateral Scler Other Motor Neuron Disord 6:131-44. 2005
    ..This review focuses on recent progress on the molecular dynamics of AMPA receptors and discusses the pathophysiology of selective motor neuron death mediated by AMPA receptors in individuals affected with sporadic ALS...
  29. ncbi request reprint Quantitative evaluation of the pyramidal tract segmented by diffusion tensor tractography: feasibility study in patients with amyotrophic lateral sclerosis
    Shigeki Aoki
    Department of Radiology, University of Tokyo Hospital, Tokyo 113 8655, Japan
    Radiat Med 23:195-9. 2005
    ..The purpose of this study was to assess the clinical feasibility of ROI analysis using diffusion tensor tractography (DTT) in patients with amyotrophic lateral sclerosis (ALS)...