FacultyDivision of Neurology Medicine
Last Updated: Dec. 06, 2017 at 12:36

Researcher Profile & Settings



Affiliation (Master)

  • Division of Neurology Medicine


  • 2000Kyoto University Graduate School, Division of Medicine
  • 1991Kyoto University Faculty of Medicine


  • MD, PhD(Kyoto University)

Association Memberships


Academic & Professional Experience

  • Jul. 2016- TodayProfessor and Chairman, Shiga University of Medical Science
  • Oct. 2013Jun. 2016Associate Professor, Kyoto University Graduate School of Medicine
  • Jun. 2009Oct. 2013Associate Professor, Shiga University of Medical Science
  • Sep. 2006Jun. 2009Assistant Professor, Shiga University of Medical Science
  • Sep. 2003Aug. 2006Postdoctoral fellow, Laval University
  • Apr. 2000Aug. 2003Staff scientist, RIKEN

Research Activities

Research Areas

  • Neuroscience / Neurochemistry/Neuropharmacology
  • Biological science / Cell biology
  • Clinical internal medicine / Neurology

Research Interests


Published Papers

  • Velocity of intraneural blood flow is increased in inflammatory neuropathies: sonographic observation.
    Carandang MA, Takamatsu N, Nodera H, Mori A, Mimura N, Okada N, Kinoshita H, Kuzuya A, Urushitani M, Takahashi R, Izumi Y, Kaji R
    Journal of neurology, neurosurgery, and psychiatry Nov. 2016 [査読有り]
  • Localization of a mutant SOD1 protein in E40K-heterozygous dogs: Implications for non-cell-autonomous pathogenesis of degenerative myelopathy.
    Kobatake Y, Sakai H, Tsukui T, Yamato O, Kohyama M, Sasaki J, Kato S, Urushitani M, Maeda S, Kamishina H
    Journal of the neurological sciences Oct. 2016 [査読有り]
  • Activated caspase-9 immunoreactivity in glial and neuronal cytoplasmic inclusions in multiple system atrophy.
    Kawamoto Y, Ayaki T, Urushitani M, Ito H, Takahashi R
    Neuroscience letters 628 207-212 Jun. 2016 [査読有り]
  • CUL2-mediated clearance of misfolded TDP-43 is paradoxically affected by VHL in oligodendrocytes in ALS.
    Uchida T, Tamaki Y, Ayaki T, Shodai A, Kaji S, Morimura T, Banno Y, Nishitsuji K, Sakashita N, Maki T, Yamashita H, Ito H, Takahashi R, Urushitani M
    Scientific reports 6 19118 Jan. 2016 [査読有り]
  • Gradual cerebral hypoperfusion in spontaneously hypertensive rats induces slowly evolving white matter abnormalities and impairs working memory.
    Kitamura A, Saito S, Maki T, Oishi N, Ayaki T, Hattori Y, Yamamoto Y, Urushitani M, Kalaria RN, Fukuyama H, Horsburgh K, Takahashi R, Ihara M
    Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism Oct. 2015 [査読有り]
  • Accumulation and aggregate formation of mutant superoxide dismutase 1 in canine degenerative myelopathy.
    Nakamae S, Kobatake Y, Suzuki R, Tsukui T, Kato S, Yamato O, Sakai H, Urushitani M, Maeda S, Kamishina H
    Neuroscience Jul. 2015 [査読有り]
  • A cysteine residue affects the conformational state and neuronal toxicity of mutant SOD1 in mice - Relevance to the pathogenesis of ALS.
    Nagano S, Takahashi Y, Yamamoto K, Masutani H, Fujiwara N, Urushitani M, Araki T
    Human molecular genetics Mar. 2015 [査読有り]
  • Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant.
    Ayaki T, Ito H, Fukushima H, Inoue T, Kondo T, Ikemoto A, Asano T, Shodai A, Fujita T, Fukui S, Morino H, Nakano S, Kusaka H, Yamashita H, Ihara M, Matsumoto R, Kawamata J, Urushitani M, Kawakami H, Takahashi R
    Acta neuropathologica communications 2 172 Dec. 2014 [査読有り]
  • Proteasome dysfunction induces muscle growth defects and protein aggregation.
    Kitajima Y, Tashiro Y, Suzuki N, Warita H, Kato M, Tateyama M, Ando R, Izumi R, Yamazaki M, Abe M, Sakimura K, Ito H, Urushitani M, Nagatomi R, Takahashi R, Aoki M
    Journal of cell science Nov. 2014 [査読有り]
  • Attenuation of endoplasmic reticulum stress in Pelizaeus-Merzbacher disease by an anti-malaria drug, chloroquine.
    Morimura T, Numata Y, Nakamura S, Hirano E, Gotoh L, Goto YI, Urushitani M, Inoue K
    Experimental biology and medicine (Maywood, N.J.) Feb. 2014 [査読有り]
  • Transglutaminase 2 accelerates neuroinflammation in amyotrophic lateral sclerosis through interaction with misfolded superoxide dismutase 1.
    Oono M, Okado-Matsumoto A, Shodai A, Ido A, Ohta Y, Abe K, Ayaki T, Ito H, Takahashi R, Taniguchi N, Urushitani M
    Journal of neurochemistry 128(3) 403-418 2014 [査読有り]
  • Aberrant assembly of RNA recognition motif 1 links to pathogenic conversion of TAR DNA-binding protein of 43 kDa (TDP-43).
    Shodai A, Morimura T, Ido A, Uchida T, Ayaki T, Takahashi R, Kitazawa S, Suzuki S, Shirouzu M, Kigawa T, Muto Y, Yokoyama S, Takahashi R, Kitahara R, Ito H, Fujiwara N, Urushitani M
    The Journal of biological chemistry 288 14886-14905 May 2013 [査読有り]
  • Motor neuron-specific disruption of proteasomes, but not autophagy, replicates amyotrophic lateral sclerosis.
    Tashiro Y, Urushitani M, Inoue H, Koike M, Uchiyama Y, Komatsu M, Tanaka K, Yamazaki M, Abe M, Misawa H, Sakimura K, Ito H, Takahashi R
    The Journal of biological chemistry 287 42984-42994 Dec. 2012 [査読有り]
  • Conserved acidic amino acid residues in a second RNA recognition motif regulate assembly and function of TDP-43.
    Shodai A, Ido A, Fujiwara N, Ayaki T, Morimura T, Oono M, Uchida T, Takahashi R, Ito H, Urushitani M
    PloS one 7 e52776 2012 [査読有り]
  • An autopsy case of SOD1-related ALS with TDP-43 positive inclusions.
    Okamoto Y, Ihara M, Urushitani M, Yamashita H, Kondo T, Tanigaki A, Oono M, Kawamata J, Ikemoto A, Kawamoto Y, Takahashi R, Ito H
    Neurology 77 1993-1995 Nov. 2011 [査読有り]
  • In vivo detection of amyloid β deposition using ¹⁹F magnetic resonance imaging with a ¹⁹F-containing curcumin derivative in a mouse model of Alzheimer's disease.
    Yanagisawa D, Amatsubo T, Morikawa S, Taguchi H, Urushitani M, Shirai N, Hirao K, Shiino A, Inubushi T, Tooyama I
    Neuroscience 184 120-127 Jun. 2011 [査読有り]
  • Colocalization of 14-3-3 proteins with SOD1 in Lewy body-like hyaline inclusions in familial amyotrophic lateral sclerosis cases and the animal model.
    Okamoto Y, Shirakashi Y, Ihara M, Urushitani M, Oono M, Kawamoto Y, Yamashita H, Shimohama S, Kato S, Hirano A, Tomimoto H, Ito H, Takahashi R
    PloS one 6 e20427 2011 [査読有り]
  • Protein Misdirection Inside and Outside Motor Neurons in Amyotrophic Lateral Sclerosis (ALS): A Possible Clue for Therapeutic Strategies.
    Ido A, Fukuyama H, Urushitani M
    International journal of molecular sciences 12 6980-7003 2011 [査読有り]
  • Induction of protective immunity by vaccination with wild-type apo superoxide dismutase 1 in mutant SOD1 transgenic mice.
    Takeuchi S, Fujiwara N, Ido A, Oono M, Takeuchi Y, Tateno M, Suzuki K, Takahashi R, Tooyama I, Taniguchi N, Julien JP, Urushitani M
    Journal of neuropathology and experimental neurology 69 1044-1056 Oct. 2010 [査読有り]

Conference Activities & Talks

  • Impaired VHL/CUL2-mediated clearance of misfolded TDP-43 underlies glial cytoplasmic inclusions (GCIs) in ALS
    urumakoto May 2016
  • Molecular epitope identifi cation for misfolded/unfolded TDP-43 in ALS
    10th Annual Symposium of ALS of the Foundation Andre-Delambre Sep. 2014
  • The role of conformation of RNA recognition motifs of TDP-43 in the protein quality control system in ALS
    6th Japan-Canada joint symposium. 37th Annual Meeting of Japan Neuroscience Society Sep. 2014
  • Molecular targeting strategies against misfolded proteins in ALS
    7th ALS Research Forum, ALS Society of Canada May 2011
  • Immunotherapy in ALS
    Neuro2010 Sep. 2010


  • Induction of protective immunity by vaccination with wild-type apo SOD1 in the mutant SOD1 transgenic mice
    J Neuropathol Exp Neurol 69,1044-1056 2010
  • Neuronal overexpression of chromogranin A accelerates disease onset in a mouse model of ALS
    J Neurochem 115,1102-1111 2010
  • Synergistic effect between proteasome and autophagosome in the clearance of poly-ubiquitinated TDP-43
    J Neurosci Res 88,784-797 2010
  • Extracellular mutant SOD1 induces microglial-mediated motoneuron injury
    Glia 58,231-243 2010
  • Relationship between the tautomeric structures of curcumin derivatives and their Abeta-binding activities in the context of therapies for Alzheimer's disease
    Biomaterials 31,4179-4185 2010
  • Chromogranin B P413L variant as risk factor and modifier of disease onset for amyotrophic lateral sclerosis.
    Proc Natl Acad Sci U S A 106,21777-21782 2009
  • Axonal ligation induces transient redistribution of TDP-43 in brainstem motor neurons
    Neuroscience 164(4) 1565-1578 2009
  • Trifluoromethoxy-benzylated ligands improve amyloid detection in the brain using (19)F magnetic resonance imaging.
  • Als2 mRNA splicing variants detected in KO mice rescue severe motor dysfunction phenotype in Als2 knock down zebrafish
    Hum Mol Genet 17(17) 2691-2702 2008
  • The endoplasmic reticulum-Golgi pathway is a target for translocation and aggregation of mutant superoxide dismutase linked to ALS
    FASEB J 22(7) 2476-2487 2008
  • Therapeutic effects of immunization with mutant superoxide dismutase in mice models of ALS
    Proc Natl Acad Sci U S A 104(7) 2495-2500 2007
  • Wild-type SOD1 acquires properties of ALS-linked SOD1 mutants through oxidation
    J Neurochem 102,170-178 2007
  • Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis
    Nat Neurosci 1,108-118 2006
  • In vivo evidence of CHIP up-regulation attenuating tau aggregation
    J Neurochem 94,1254-1263 2005
  • siRNA-based inhibition specific for mutant SOD1 with single nucleotide alteration in familial ALS, compared with ribozyme and DNA enzyme
    Biochem Biophys Res Commun 314,283-291 2004
  • CHIP promotes proteasomal degradation of familial ALS-linked mutant SOD1 by ubiquitinating Hsc/Hsp70
    J Neurochem 90,231-244 2004
  • Calcium-permeable AMPA receptors promote misfolding of mutant SOD1 protein and development of amyotrophic lateral sclerosis in a transgenic mouse model
    Hum Mol Genet 13,2183-2196 2004
  • Proteasomal inhibition by misfolded mutant superoxide dismutase 1 induces selective motor neuron death in familial amyotrophic lateral sclerosis
    J Neurochem 83,1030-1041 2002
  • NMDA receptor-mediated mitochondrial Ca2+ overload in acute excitotoxic motor neuron death; a distinct mechanism from chronic neurotoxicity after Ca2+ influx
    J Neurosci Res 63,377-387 2001
  • The role of nitric oxide in amyotrophic lateral sclerosis
    Amyot Lat Scler Other Motor Neuron Disord 2(2) 71-82 2001
  • Neuroprotective mechanism of GDNF in mesencephalic neurons
    J Neurochem 74,1175-1184 2000
  • Mechanisms of antiapoptotic effects of estrogens in nigral dopaminergic neurons
    FASEB J 14,1202-1214 2000
  • Phosphatidylinositol 3-kinase mediates neuroprotection by estrogen in cultured cortical neurons
    J Neurosci Res 60,321-327 2000
  • Neuroprotective effect of cyclic GMP against radical-induced toxicity in cultured spinal motor neurons
    J Neurosci Res 61,443-448 2000
  • Protection of cultured spinal motor neurons by estradiol
    Neuroreport 11,3493-3497 2000
  • Stimulation of alpha4beta2 nicotinic acetylcholine receptors inhibits beta-amyloid toxicity
    Brain Res 792,331-334 1998
  • Dopamine D2-type agonists protect mesencephalic neurons from glutamate neurotoxicity: mechanisms of neuroprotective treatment against oxidative stress
    Ann Neurol 44,110-119 1998
  • Mechanism of selective motor neuronal death after exposure of spinal cord to glutamate: involvement of glutamate-induced nitric oxide in motor neuron toxicity and nonmotor neuron protection
    Ann Neurol 44,796-807 1998
  • Estradiol protects mesencephalic dopaminergic neurons from oxidative stress-induced neuronal death
    J Neurosci Res 54,707-719 1998
  • MRI demonstration of a reversible lesion in cerebral deep white matter in thrombotic thrombocytopenic purpura
    Neuroradiology 38,137-138 1996
  • Miller Fisher-Guillain-Barre overlap syndrome with enhancing lesions in the spinocerebellar tracts
    J Neurol Neurosurg Psychiatry 58,241-243 1995

Awards & Honors

  • 2004 Uehara Memorial Foundation Postdoctoral fellowship

Research Grants & Projects

  • Identifacation of disease-specific molecular target to develop a novel therapy against neurodegenerative diseases
    :R and D Consignment Program
  • A novel strategy of ALS research by investigating the sructural instability of TDP-43
    :Grant-in-Aid for Scientific Research

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