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  1. Watanabe M, Nakamura Y, Sato S, Niino M, Fukaura H, Tanaka M, Ochi H, Kanda T, Takeshita Y, Yokota T, Nishida Y, Matsui M, Nagayama S, Kusunoki S, Miyamoto K, Mizuno M, Kawachi I, Saji E, Ohashi T, Shimohama S, Hisahara S, Nishiyama K, Iizuka T, Nakatsuji Y, Okuno T, Ochi K, Suzumura A, Yamamoto K, Kawano Y, Tsuji S, Hirata M, Sakate R, Kimura T, Shimizu Y, Nagaishi A, Okada K, Hayashi F, Sakoda A, Masaki K, Shinoda K, Isobe N, Matsushita T, Kira JI. HLA genotype-clinical phenotype correlations in multiple sclerosis and neuromyelitis optica spectrum disorders based on Japan MS/NMOSD Biobank data. Sci Rep 11(1):607, 2021.


  1. Okada K, Kakeda S, Tahara M. Olfactory identification associates with cognitive function and the third ventricle width in patients with relapsing-remitting multiple sclerosis. Mult Scler Relat Disord 38:101507, 2020.
  2. Miyata M, Kakeda S, Hashimoto T, Ide S, Okada K, Adachi H, Korogi Y. Facial nerve atrophy in patients with amyotrophic lateral sclerosis: Evaluation withfastimaging employing steady-state acquisition (FIESTA). J Magn Reson Imaging 51(3):757-766, 2020.
  3. Halievski K, Xu Y, Haddad YW, Tang YP, Yamada S, Katsuno M, Adachi H, Sobue G, Breedlove SM, Jordan CL. Muscle BDNF improves synaptic and contractile muscle strength in Kennedy's disease mice in a muscle-type specific manner. J Physiol 598: 2719-2739, 2020.
  4. Masaki H, Watanabe K, Kakeda S, Ide S, Sugimoto K, Ueda I, Hamamura T, Hisanaga S, Toyota T, Akamatsu N, Shimajiri S, Yamamoto J, Nishizawa S, Adachi H, Korogi Y. Hippocampal sclerosis without visually detectable hippocampal MRI abnormalities: automated subfield volumetric analysis. Jpn J Radiol 38(11):1020-1027, 2020.
  5. Tahara M, Oeda T, Okada K, Kiriyama T, Ochi K, Maruyama H, Fukaura H, Nomura K, Shimizu Y, Mori M, Nakashima I, Misu T, Umemura A, Yamamoto K, Sawada H. Safety and efficacy of rituximab in neuromyelitis optica spectrum disorders (RIN-1 study): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol 19:298-306, 2020.


  1. Okada K, Kobata M, Naruke S. Neuromyelitis optica spectrum disorder with area postrema syndrome. Neurol Clin Pract 9:173-175, 2019.
  2. Mizuguchi T, Toyota T, Adachi H, Miyake N, Matsumoto N, Miyatake S. Detecting a long insertion variant in SAMD12 by SMRT sequencing: implications of long-read whole-genome sequencing for repeat expansion diseases. J Hum Genet 64: 191-197, 2019.
  3. Halievski K, Nath SR, Katsuno M, Adachi H, Sobue G, Breedlove M, Lieberman AP, Jordan CL. Disease affects Bdnf expression in synaptic and extrasynaptic regions of skeletal muscle of three SBMA mouse models. Int J Mol Sci 20, E1314, 2019.
  4. Mitsuhashi S, Frith MC, Mizuguchi T, Miyatake S, Toyota T, Adachi H, Oma Y, Kino Y, Mitsuhashi H, Matsumoto N. Tandem-genotypes: Robust detection of tandem repeat expansions from long DNA reads. Genome Biol 20:58, 2019.
  5. Kondo N, Tohnai G, Sahashi K, Iida M, Kataoka M, Nakatsuji H, Tsutsumi Y, Hashizume A, Adachi H, Koike H, Shinjo K, Kondo Y, Sobue G, Katsuno M. DNA methylation inhibitor attenuates polyglutamine-induced neurodegeneration by regulating Hes5. EMBO mol med 11, e8547, 2019.
  6. Ide S, Kakeda S, Adachi H, Miyata M, Iwanaka Y, Okada K, Korogi Y. Detection of dentate nuclei abnormality in a patient with dentatorubral-pallidoluysian atrophy using the quantitative susceptibility mapping. J Neurol Sci 403 97-98, 2019.
  7. Miyata M, Kakeda S, Toyoshima Y, Ide S, Okada K, Adachi H, Wang Y, Korogi Y. Potential usefulness of signal intensity of cerebral gyri on quantitative susceptibility mapping for discriminating corticobasal degeneration from progressive supranuclear palsy and Parkinson's disease. Neuroradiology 61: 1251-1259, 2019.
  8. Iida M, Sahashi K, Kondo N, Nakatsuji H, Tohnai G, Tsutsumi Y, Adachi H, Ishigaki S, Fujioka Y, Watanabe H, Tanaka F, Nakai A, Sobue G, Katsuno M. Src inhibition attenuates polyglutamine-mediated neuromuscular degeneration in spinal and bulbar muscular atrophy. Nat Commun 10:4262, 2019.
  9. Miyata M, Kakeda S, Hashimoto T, Korogi Y, Adachi H. The facial nerve atrophy with spinal and bulbar muscular atrophy patients (SBMA): three case reports with 3D fast imaging employing steady-state acquisition (FIESTA). J Neurol Sci 406:116461, 2019.


  1. Okada K, Hashimoto T, Kobata M, Kakeda S, Takahashi T, Hirato J. Tumefactive demyelination with a transient appearance of oligoclonal bands in MS under fingolimod. Neurol Neuroimmunol Neuroinflamm 5:e484, 2018.
  2. Ninomiya H, Ohgami N, Oshino R, Kato M, Ohgami K, Li X, Shen D, Iida M, Yajima I, Angelidis CE, Adachi H, Katsuno M, Sobue G, Kato M. Increased expression level of Hsp70 in the inner ears of mice by exposure to low frequency noise. Hear Res 363: 49-54, 2018.
  3. Xu Y, Halievski K, Katsuno M, Adachi H, Sobue G, Breedlove M, Jordan CL. Pre-clinical symptoms of SBMA may not be androgen-dependent: Implications from two SBMA mouse models. Hum Mol Genet 27: 2425-2442, 2018.
  4. Mitsui S, Otomo A, Nozaki M, Ono S, Sato K, Shirakawa R, Adachi H, Aoki M, Sobue G, Shang H-Fand, Hadano S. Systemic overexpression of SQSTM1/p62 accelerates disease onset in a SOD1H46R-expressing ALS mouse model. Mol Brain 11:30, 2018.
  5. Hashimoto T, Sennari Y, Okada K, Adachi H. Cerebrotendinous Xanthomatosis with Nodular-hypertrophy of the Lumbosacral Roots. Intern Med 57:1669-1670, 2018.
  6. Ohnari K, Okada K, Higuchi O, Matsuo H, Adachi H. Late-onset Myasthenia Gravis Accompanied by Amyotrophic Lateral Sclerosis with Antibodies against the Acetylcholine Receptor and Low-density Lipoprotein Receptor-related Protein 4. Intern Med 57:3021-3024, 2018.


  1. Kuwabara S, Mori M, Misawa S, Suzuki M, Nishiyama K, Mutoh T, Doi S, Kokubun N, Kamijo M, Yoshikawa H, Abe K, Nishida Y, Okada K, Sekiguchi K, Sakamoto K, Kusunoki S, Sobue G, Kaji R; Glovenin-I CIDP Study Group.Intravenous immunoglobulin for maintenance treatment of chronic inflammatory demyelinating polyneuropathy: a multicentre, open-label, 52-week phase III trial. J Neurol Neurosurg Psychiatry 88:832-838, 2017.
  2. Okada K, Kobata M, Sennari Y, Iwanaka Y, Hashimoto T, Ohnari K, Tahara M.Levels of nitric oxide metabolites in cerebrospinal fluid correlate with cognitive impairment in early stage multiple sclerosis. J Neurol Neurosurg Psychiatry 88:892-893, 2017.
  3. Toyota T, Sennari Y, Okada K, Kawahara A, Tsukada J, Shimajiri S, Adachi H. Myoclonia continua in primary central nervous system natural killer/T-cell lymphoma, nasal type. Neurology 88: 329-330, 2017.
  4. Kobori S, Kubo T, Otani M, Muramatsu K, Fujino Y, Adachi H, Horiguchi H, Fushimi K, Matsuda S. Coexisting infectious diseases on admission as a risk factor for mechanical ventilation in patients with Guillain–Barré syndrome.J Epidemiol 27: 311-316, 2017.


  1. Toyota T, Akamatsu N, Tanaka A, Yamano M, Fujino Y, Adachi H. Interictal epileptiform discharges in new-onset epilepsy among older adults. Neurol Clin Neurosci 4: 3-9, 2016.
  2. Kakeda S, Yoneda T, Ide S, Miyata M, Hashimoto T, Futatsuya K, Watanabe K, Murakami Y, Ogasawara A, Moriya J, Sato T, Okada K, Uozumi T, Adachi H, Korogi Y. Zebra sign of precentral gyri in amyotrophic lateral sclerosis: A novel finding using phase difference enhanced (PADRE) imaging. Eur Radiol  26:4173-4183, 2016.
  3. Futatsuya K, Kakeda S, Yoneda T, Ueda I, Watanabe K, Moriya J, Murakami Y, Ide S, Ogasawara A, Ohnari N, Okada K, Adachi H, Korogi Y. Juxtacortical lesions in multiple sclerosis: Assessment of gray matter involvement with phase difference-enhanced imaging (PADRE). Magn Reson Med Sci 15:349-354, 2016.
  4. Ohnari K, Okada K, Takahashi T, Mafune K, Adachi H. Evoked potentials are useful for diagnosis of neuromyelitis optica spectrum disorder. J Neurol Sci 364: 97-101, 2016.
  5. Onozato T, Nakahara A, Suzuki-Kouyama E, Hineno A, Yasude T, Nakamura T, Yahikozawa H, Watanabe M, Kayanuma K, Makishita H, Ohara S, Hashimoto T, Higuchi K, Sakai T, Asano K, Hashimoto T, Kanno H, Nakayama J, Oyanagi K. Axonal TDP-43 aggregates in sporadic amyotrophic lateral sclerosis. Neuropathol Appl Neurobiol 42:561-572, 2016.
  6. Xu Y, Halievski K, Henley C, Atchison W, Katsuno M, Adachi H, Sobue G, Breedlove S, Jordan CL. Defects in neuromuscular transmission may underlie motor dysfunction in SBMA. J Neurosci 36: 5094-5106, 2016.
  7. Ding Y, Adachi H, Katsuno M, Sahashi K, Kondo N, Iida M, Tohnai G, Nakatsuji H, Sobue G. BIIB021, a synthetic Hsp90 inhibitor, induces mutant ataxin-1 degradation through the activation of heat shock factor 1. Neuroscience 327:20-31, 2016.
  8. Hijikata Y, Katsuno M, Suzuki K, Hashizume A, Araki A, Yamada S, Inagaki T, Iida M, Noda S, Nakanishi H, Banno H, Mano T, Hirakawa A, Adachi H, Watanabe H, Yamamoto M, Sobue G. Impaired muscle uptake of creatine in spinal and bulbar muscular atrophy. Ann Clin Transl Neurol 3:537-546, 2016.
  9. Huang Z, Adachi H. Natural Compounds Preventing Neurodegenerative Diseases Through Autophagic Activation. J UOEH 38:139-48, 2016.
  10. Kino Y, Washizu C, Kurosawa M, Yamada M, Doi H, Takumi T, Adachi H, Katsuno M, Sobue G, Hicks G, Hattori N, Shimogori T, Nukina N. FUS/TLS acts as an aggregation-dependent modifier of polyglutamine disease model mice. Sci Rep 6: 35236, 2016.
  11. Okada K. Cerebrospinal fluid/serum immunoglobulin G quotient is predictive of unresponsiveness to intravenous methylprednisolone therapy for acute exacerbation of neuromyelitis optica spectrum disorder. Clin Exp Neuroimmunol 7: 209-210, 2016.

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  1. Iida M, Katsuno M, Nakatsuji H, Adachi H, Kondo N, Miyazaki Y, Tohnai G, Ikenaka K, Watanabe H, Yamamoto M, Kishida K, Sobue G.Pioglitazone suppresses neuronal and muscular degeneration caused by polyglutamine-expanded androgen receptors. Hum Mol Genet 24:314-329, 2015.
  2. Oki K, Halievski K, Vicente L, Xu Y, Zeolla D, Poort J, Katsuno M, Adachi H, Sobue G, Wiseman RW, Breedlove SM, Jordan CL. Contractile dysfunction in muscle may underlie androgen-dependent motor dysfunction in SBMA. J Appl Physiol 118: 941-952, 2015.
  3. Kawagashira Y, Koike H, Ohyama K, Hashimoto R, Iijima M, Adachi H, Katsuno M, Chapman M, Lunn M, Sobue G.Axonal loss influences the response to rituximab treatment in neuropathy associated with IgM monoclonal gammopathy with anti-myelin-associated glycoprotein antibody. J Neurol Sci 348: 67-73, 2015.
  4. Halievski K, Henley CL, Domino L, Poort JE, Fu M, Katsuno M, Adachi H, Sobue G, Breedlove SM, Jordan CL. Androgen-dependent loss of muscle BDNF mRNA in two mouse models of SBMA. Exp Neurol 269: 224-232, 2015.
  5. Okada K, Adachi H. Human immunoglobulin G suppresses the production of matrix metalloproteinase-9 in peripheral blood mononuclear cells of patients with multiple sclerosis. Clin Exp Neuroimmunol DOI: 10.1111/cen3.12214
  6. Sahashi K, Katsuno M, Hung G, Adachi H, Kondo N, Nakatsuji H, Tohnai G, Iida M, Bennett F, Sobue G. Silencing neuronal mutant androgen receptor in a mouse model of spinal and bulbar muscular atrophy. Hum Mol Gent24: 5985-5994, 2015.
  7. Kobata M, Okada K, Hashimoto T, Matsuyama A, Uchibori A, Takahashi T, Adachi H. Paraneoplastic neuromyelitis optica spectrum disorder manifesting as intractable nausea and acute cerebellar ataxia associated with lung adenocarcinoma. Neurol Clin Neurosci 6: 223-225, 2015.
  8. Toyota T, Huang Z, Nohara S, Okada K, Kakeda S, Korogi Y,Nakayama T, Sone J, SobueG, Adachi H. Neuronal intranuclear inclusion disease manifesting with new-onset epilepsy in the elderly. Neurol Clin Neurosci 6: 238-240, 2015.
  9. Ding Y, Adachi H, Katsuno M, Huang Z, Jiang YM, Kondo N, Iida M, Tohnai G, Nakatsuji H, Funakoshi H, Nakamura T, Sobue G. Overexpression of hepatocyte growth factor in SBMA model mice has an additive effect on combination therapy with castration. Biochem Biophys Res Commun 468: 677-683, 2015.
  10. Oyanagi K, Yamazaki M, Hashimoto T, Asakawa M, Wakabayashi K, Takahashi H. Hippocampal sclerosis in the parkinsonism-dementia complex of Guam: quantitative examination of neurons, neurofibrillary tangles, and TDP-43 immunoreactivity in CA1. Neuropathology 35:224-235, 2015.
  11. Oyanagi K, Mochizuki Y, Nakayama Y, Hayashi K, Shimizu T, Nagao M, Hashimoto T, Yamazaki M, Matsubara S, Komori T. Marked preservation of the visual and olfactory pathways in ALS patients in a totally locked-in state. Clin Neuropathol 34:267-274, 2015.

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  1. Renier KJ, Troxell-Smith SM, Johansen JA, Katsuno M, Adachi H, Sobue G, Chua JP, Sun Kim H, Lieberman AP, Breedlove SM, Jordan CL. Anti-androgen flutamide protects male mice from androgen-dependent toxicity in three models of spinal bulbar muscular atrophy. Endocrinology 155: 2624-2634, 2014.
  2. Ohyama K, Koike H, Katsuno M, Takahashi M, Hashimoto R, Kawagashira Y, Iijima M, Adachi H, Watanabe H, Sobue G. Muscle atrophy in chronic inflammatory demyelinating polyneuropathy: a computed tomography assessment. Eur J Neurol 21: 1002-1010, 2014.
  3. Tohnai G, Adachi H, Katsuno M, Doi H, Matsumoto S, Kondo N, Miyazaki Y, Iida M, Nakatsuji H, Qiang Q, Ding Y, Watanabe H, Yamamoto M, Ohtsuka K, Sobue G. Paeoniflorin eliminates a mutant AR via NF-YA-dependent proteolysis in spinal and bulbar muscular atrophy. Hum Mol Genet 23: 3552-3565, 2014.
  4. Toyota T,Akamatsu N,Tanaka A,Tsuji S,Uozumi T:Electroencephalographic features of benign adult familial myoclonic epilepsy.Clin Neurophysiol 125:250-254,2014.
  5. Tanaka S, Banno H, Katsuno M, Suzuki K, Suga N, Hashizume A, Mano T, Araki A, Watanabe H, Adachi H, Tatsumi H, Yamamoto M, Sobue G. Distinct acoustic features in spinal and bulbar muscular atrophy patients with laryngospasm. J Neurol Sci 337:193-200, 2014.
  6. Hara K, Watanabe H, Ito M, Tsuboi T, Watanabe H, Nakamura R, Senda J, Atsuta N, Adachi H, Aiba I, Naganawa S, Sobue G. Potential of a new MRI for visualizing cerebellar involvement in progressive supranuclear palsy. Parkinsonism Relat Disord 20: 157-161, 2014
  7. Chua JP, Reddy SL, Merry DE, Adachi H, Katsuno M, Sobue G, Robins DM, Lieberman AP. Transcriptional activation of TFEB/ZKSCAN3 target genes underlies enhanced autophagy in spinobulbar muscular atrophy. Hum Mol Genet 23:1376-1386, 2014.
  8. Toyota T, Akamatsu N, Tsuji S, Nishizawa S. Limbic encephalitis associated with anti-voltage-gated potassium channel complex antibodies as a cause of adult-onset mesial temporal lobe epilepsy. J UOEH 36:129-33, 2014.
  9. Mochizuki Y, Kawata A, Hashimoto T, Akiyama H, Kawakami H, Komori T, Oyanagi K, Mizutani T, Matsubara S. An autopsy case of familial amyotrophic lateral sclerosis with FUS R521G mutation. Amyotroph Lateral Scler Frontotemporal Degener 15:305-308,2014.

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  1. Watanabe H, Senda J, Kato S, Ito M, Atsuta N, Hara K, Tsuboi T, Katsuno M, Nakamura T, Hirayama M, Adachi H, Naganawa S, Sobue G. Cortical and subcortical brain atrophy in Parkinson's disease with visual hallucination. Mov Disord 28:1732-1736, 2013.
  2. Tanaka A, Akamatsu N, Shouzaki T, Toyota T, Yamano M, Nakagawa M, Tsuji S: Clinical characteristics and treatment responses in new-onset epilepsy in the elderly. Seizure 22:772-775, 2013.
  3. Kakeda S, Korogi Y, Yoneda T, Watanabe K, Moriya J, Murakami Y, Sato T, Hiai Y, Ohnari N, Ide S,Okada K, Uozumi T, Tsuji S, Hirai T, Yamashita Y. Parkinson's disease: diagnostic potential of high-resolution phase difference enhanced MR imaging at 3 T. Eur Radiol 23:1102-1111, 2013.
  4. Tomita M, Koike H, Kawagashira Y, Iijima M, Adachi H, Taguchi J, Abe T, Sako K, Tsuji Y, Nakagawa M, Kanda F, Takeda F, Sugawara M, Toyoshima I, Asano N, Sobue G. Clinicopathological features of neuropathy associated with lymphoma. Brain 136(Pt 8):2563-2578, 2013.
  5. Takagi S, Iguchi Y, Katsuno M,Ishigaki S, Ikenaka K, Fujiok Y, Honda D, Niwa J, Tanaka F, Watanabe H, Adachi H, Sobue G. RNP2 of RNA recognition motif 1 plays a central role in the aberrant modification of TDP-43. PLOS ONE 8:e66966, 2013. (PMID: 23840565)
  6. Yang YC, Fu HC, Hsiao BL, Sobue G, Adachi H, Huang FJ, Hsuuw YD, Wei KT, Chang C, Huang KE, Kang HY. Androgen receptor inclusions acquire GRP78/BiP to ameliorate androgen-induced protein misfolding stress in embryonic stem cells. Cell Death Dis 4:e607, 2013. doi: 10.1038/cddis.2013.122. (PMID: 23618905)
  7. Doi H*, Adachi H*, Katsuno M, Minamiyama M, Matsumoto S,Kondo N, Miyazaki Y, Iida M, Tohnai G, Qiang Q, Tanaka F, Yanagawa T, Warabi E, Ishii T, Sobue G. p62/SQSTM1 differentially removes the toxic mutant androgen receptor via autophagy and inclusion formation in a spinal and bulbar muscular atrophy mouse model. J Neurosci 33: 7710-7727, 2013. (* Evenly contributed to the study)
  8. Qiang Q, Adachi H, Huang Z, Jiang YM, Katsuno M, Minamiyama M, Doi H, Matsumoto S, Kondo N, Miyazaki Y, Iida M, Tohnai G, Sobue G. Genistein, a natural product derived from soybeans, ameliorates polyglutamine-mediated motor neuron disease. J Neurochem 126:122-130, 2013.
  9. Kondo N, Katsuno M, Adachi H, Minamiyama M, Doi H, Matsumoto S, Miyazaki Y, Iida M, Tohnai G, Nakatsuji H, Ishigaki S, Fujioka Y, Watanabe H, Tanaka F, Nakai A, Sobue G. Heat shock factor-1 influences pathological lesion distribution of polyglutamine-induced neurodegeneration. Nat Commun 4: 1405 doi:10.1038/ncomms2417, 2013.
  10. Matsuda M, Hoshino T, Yamakawa N, Tahara K, Adachi H, Sobue G, Maji D, Ihn H, Mizushima T. Suppression of UV-Induced Wrinkle Formation by Induction of HSP70 Expression in Mice. J Invest Dermatol 133: 919-928,2013.
  11. Tanaka A,Akamatsu N,Yamano M,Nakagawa M,Kawamura M,Tsuji S:A more realistic approach, using dynamic stimuli, to test facialemotion recognition impairment in temporal lobe epilepsy.Epilepsy Behav 28:12-16,2013.
  12. Toyota T,Akamatsu N,Tanaka A,Shouzaki T,Tsuji S,Saito K,Tanaka Y: Mesial temporal lobe epilepsy as a neuropsychiatric syndrome of systemic lupus erythematosus.Epilepsia 54:e33-e36,2013.

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  1. Katsumata R, Ishigaki S, Katsuno M, Kawai K, Sone J, Huang Z, Adachi H, Tanaka F, Urano F, Sobue G. c-Abl Inhibition Delays Motor Neuron Dege neration in the G93A Mouse, an Animal Model of Amyotrophic Lateral Sclerosis. PLOS ONE 7:e46185, 2012.
  2. Minamiyama M, Katsuno M, Adachi H, Doi H, Kondo N, Iida M, Ishigaki S, Fujioka Y, Matsumoto S, Miyazaki Y, Tanaka F, Kurihara H, Sobue G. Naratriptan mitigates CGRP1-associated motor neuron degeneration caused by expanded polyglutamine. Nat Med 18: 1531-1538,2012.
  3. Taira M, Ishiura H, Mitsui J, Takahashi Y, Hayashi T, Shimizu J, Matsukawa T, Saito N, Okada K, Tsuji S, Sawamura H, Amano S, Goto J, Tsuji S. Clinical features and haplotype analysis of newly identified Japanese patients with gelsolin-related familial amyloidosis of Finnish type. Neurogenetics 13:237-43, 2012.
  4. Katsuno M, Tanaka F, Adachi H, Banno H, Suzuki K, Watanabe H, Sobue G. Pathogenesis and therapy of spinal and bulbar muscular atrophy (SBMA). Prog Neurobiol 99:246-256, 2012.
  5. Tanaka F, Katsuno M, Banno H, Suzuki K, Adachi H, Sobue G. Current Status of Treatment of Spinal and Bulbar Muscular Atrophy. Neural Plasticity, 2012. doi:10.1155/2012/369284.
  6. Miyazaki Y, Adachi H, Katsuno M, Minamiyama M, Jiang YM, Huang Z, Doi H, Matsumoto S, Kondo N, Iida N, Tohnai G, Tanaka F, Muramatsu S, Sobue G. Viral delivery of miR-196a ameliorates the spinal and bulbar muscular atrophy phenotype via the silencing of CUGBP, Elav-like family member 2. Nat Med 18: 1136-1141, 2012.
  7. Katsuno M, Banno H, Suzuki K, Adachi H, Tanaka F, Sobue G. Molecular Pathophysiology and Disease-Modifying Therapies for Spinal and Bulbar Muscular Atrophy. Arch Neurol 69: 436-440, 2012.
  8. Mochizuki Y,Isozaki E,Takao M,Hashimoto T,Shibuya M,Arai M,Hosokawa  M,Kawata A,Oyanagi K,Mihara B,Mizutani T:Familial ALS with FUS P525L mutation:two Japanese sisters with multiple systems involvement.J Neuro Sci 323:85-92,2012.
  9. Yasuda C,Okada K,Takechi U,Tsuji S:Ring-enhanced brainstem lesion in a patient with neuro-behcet's disease. Intern Med 51:991-992,2012.
  10. Luders H.O,Tsuji S,et al:Modern technology calls for a modern approach to classification of epileptic seizures and the epilepsies.Epilepsia 53: 405-411, 2012.

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  1. Yu Z, Wang AM, Adachi H, Katsuno M, Sobue G, Yue Z, Robins DM, Lieberman AP. Macroautophagy Is Regulated by the UPR-Mediator CHOP and Accentuates the Phenotype of SBMA Mice. PLoS Genet 7:e1002321, 2011.
  2. Hoshino T, Murao N, Namba T, Takehara M, Adachi H, Katsuno M, Sobue G, Matsushima T, Suzuki T, Mizushima T. Suppression of Alzheimer's disease-related phenotypes by expression of heat shock protein 70 in mice. J Neurosci 31: 5225-5234, 2011.
  3. Kakeda S, Korogi Y, Yoneda T, Nishimura J, Sato T, Hiai Y, Ohnari N, Okada K, Hayashi H, Matsusue E, Uozumi T, Tsuji S. A novel tract imaging technique of the brainstem using phase difference enhanced imaging: normal anatomy and initial experience in multiple system atrophy. Eur Radiol 21:2202-2210, 2011.
  4. Katsuno M, Adachi H, Banno H, Suzuki K, Tanaka F, Sobue G. Transforming Growth Factor-β Signaling in Motor Neuron Diseases. Curr Mol Med 11:48-56, 2011.
  5. Cheng XW, Huang Z, Kuzuya M, Okumura K, Murohara T. Cysteine protease cathepsins in atherosclerosis-based vascular disease and its complications, Hypertension 58: 978-986, 2011.
  6. Cheng XW, Kuzuya M, Sasaki T, Inoue A, Hu L, Song H, Huang Z, Li P, Takeshita K, Hirashiki A, Sato K, Shi GP, Okumura K, Murohara T. Inhibition of mineralocorticoid receptor is a renoprotective effect of the 3-hydroxy-3-methylglutaryl-coenzyme A reductase inhibitor pitavastatin, Journal of hypertension 29: 542-552, 2011.

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  1. Mo K, Razak Z, Rao P, Yu Z, Adachi H, Katsuno M, Sobue G, Lieberman AP, Westwood JT, Monks DA. Microarray Analysis of Gene Expression by Skeletal Muscle of Three Mouse Models of Kennedy Disease/Spinal Bulbar Muscular Atrophy. PLoS ONE 5: e12922, 2010.
  2. Koike H, Atsuta N, Adachi H, Iijima M, Katsuno M, Yasuda T, Fukada Y, Yasui K, Nakashima K, Horiuchi M, Shiomi K, Fukui K, Takashima S, Morita Y, Kuniyoshi K, Hasegawa Y, Toribe Y, Kajiura M, Takeshita S, Mukai E, Sobue G. Clinicopathological features of acute autonomic and sensory neuropathy. Brain 133(10): 2881-2896, 2010.
  3. Katsuno M, Banno H, Suzuki K, Adachi H, Tanaka F, Sobue G. Clinical features and molecular mechanisms of spinal and bulbar muscular atrophy (SBMA). Adv Exp Med Biol 685:64-74, 2010.
  4. Katsuno M, Adachi H, Minamiyama M, Waza M, Doi H, Kondo N, Mizoguchi H, Nitta A, Yamada K, Banno H, Suzuki K, Tanaka F, Sobue G. Disrupted transforming growth factor-beta signaling in spinal and bulbar muscular atrophy. J Neurosci 30:5702-5712, 2010.
  5. Hoshino T, Matsuda M, Yamashita Y, Takehara M, Fukuya M, Mineda K, Maji D, Ihn H, Adachi H, Sobue G, Funasaka Y, Mizushima T. Suppression of melanin production by expression of HSP70. J Biol Chem 285:13254-13263, 2010.
  6. Matsuda M, Hoshino T, Yamashita Y, Tanaka KI, Maji D, Sato K, Adachi H, Sobue G, Ihn H, Funasaka Y, Mizushima T. Prevention of ultraviolet B radiation-induced epidermal damage by expression of heat shock protein 70. J Biol Chem 285: 5848-5858, 2010.
  7. Sone J, Niwa J, Kawai K, Ishigaki S, Yamada S, Adachi H, Katsuno M, Tanaka F, Doyu M, Sobue G. Dorfin ameliorates phenotypes in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurosci Res 88:123-135, 2010.
  8. Yamano M,Akamatsu N,Tsuji S,Kobayakawa M,Kawamura, M:Decision-making in temporal lobe epilepsy examined with the lowa Gambling Task.Epilepsy Res 93:33-38,2010.
  9. Ando T,Komaki G,Nishimura H,Naruo T,Okabe K,Kawai K.,Takii M,Oka T,Kodama N,Nakamoto C,Ishikawa T,et al:A ghrelin gene variant may predict crossover rate from restricting-type anorexia nervosa to other phenotypes of eating disorders: a retrospective survival analysis.Psychiatr Genet 20:153-159, 2010.
  10. Hayashida S,Oka T,Mera T,Tsuji S:Repeated social defeat stress induces chronic hyperthermia in rats.Physiol Behav 101:124-131,2010.
  11. Okada K,Matsushita T,Kira J,Tsuji S:B-cell activating factor of the TNF family is upregulated in neuromyelitis optica.Neurology 74:177-178,2010.
  12. Piao Y-S, Hashimoto T, Sachiko Takahama S, Kakita A, Komori T, Morita T, Takahashi H, Mizutani T, Oyanagi K. The survival motor neuron protein in the spinal anterior horn cells in patients with sporadic amyotrphic lateral sclerosis. Brain Research 1372:152-159, 2010.

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  1. Adachi H, Katsuno M, Waza M, Minamiyama M, Tanaka F, Sobue G. Heat shock proteins in neurodegenerative diseases: pathogenic roles and therapeutic implications. Int J Hyperthermia 25:647-654, 2009.
  2. Palazzolo I, Stack C, Kong L, Musaro A, Adachi H, Katsuno M, Sobue G, Taylor JP, Sumner CJ, Fischbeck KH, Pennuto M. Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy. Neuron 63:316-328, 2009.
  3. Iguchi Y, Katsuno M, Niwa JI, Yamada SI, Sone J, Waza M, Adachi H, Tanaka F, Nagata KI, Arimura N, Watanabe T, Kaibuchi K, Sobue G.TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases. J Biol Chem 284:22059-22066, 2009.
  4. Asano T, Tanaka KI, Yamakawa N, Adachi H, Sobue G, Goto H, Takeuchi K, Mizushima T. HSP70 Confers Protection against Indomethacin-Induced Lesions of the Small Intestine. J Pharmacol Exp Ther 330: 458-467, 2009.
  5. Suemasu S, Tanaka KI, Namba T, Ishihara T, Katsu T, Fujimoto M, Adachi H, Sobue G, Takeuchi K, Nakai A, Mizushima T. A role for HSP70 in protecting against indomethacin-induced gastric lesions. J Biol Chem 284:19705-19715, 2009.
  6. Katsuno M, Adachi H, Sobue G. Getting a handle on Huntington's disease: the case for cholesterol. Nat Med 15:253-254, 2009.
  7. Banno H, Katsuno M, Suzuki K, Takeuchi Y, Kawashima M, Suga N, Takamori M, Ito M, Nakamura T, Matsuo K, Yamada S, Oki Y, Adachi H, Minamiyama M, Waza M, Atsuta N, Watanabe H, Fujimoto Y, Nakashima T, Tanaka F, Doyu M, Sobue G. Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy. Ann Neurol 65:140-150, 2009.
  8. Tokui K*, Adachi H*, Waza M, Katsuno M, Minamiyama M, Doi H, Tanaka K, Hamazaki J, Murata S, Tanaka F, Sobue G. 17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in a SBMA model mouse. Hum Mol Genet 18:898-910, 2009. (* Evenly contributed to the study)
  9. Kaneda Y,Tsuji S,Oka T:Age distribution and gender differences in psychogenic fever patients.Biophychosoc Med 3:6-6,2009.
  10. Sun LY, Kosugi Y, Kawakami E, Piao Y-S, Hashimoto T, Oyanagi K. Magnesium concentration in the cerebrospinal fluid of mice and its response to changes in serum magnesium concentration.Magnesium Res22: 266-272, 2009.

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  1. Katsuno M, Banno H, Suzuki K, Takeuchi Y, Kawashima M, Tanaka F, Adachi H, Sobue G. Molecular genetics and biomarkers of polyglutamine diseases. Curr Mol Med 8:221-234, 2008.
  2. Takayanagi Y,Matsumoto H,Nakata M,Mera T,Fukusumi S,Hinuma S,Ueta Y,Yada T,Leng G,Onaka T:Endogenous prolactin-releasing peptide regulates food intake in rodents.J Clin Invest 118:4014-4024,2008.
  3. Uozumi T,Takechi U,Yoshinaga K,Tsuji S:Motor Excitability after the C Reflex in Cortical Refex Myoclonus.J UOEH 30:391-401,2008.
  4. Hamada M,Ugawa Y,Tsuji S:High-Frequency rTMS over the Supplementary Motor Area for Treatment of Parkinson's Disease.Mov Disord 23: 1524-1531,2008.
  5. Ohnari K,Aoki M,Uozumi T,Tsuji S:Severe symptoms of 16q-ADCA coexisting with SCA8 repeat expansion.J Neurol Sci 273: 15-18,2008.
  6. Hashimoto T,Nishi K,Nagasao J,Tsuji S,Oyanagi K:Magnesium exerts both preventive and ameliorating effects in an in vitro rat Parkinson disease model involving 1-methyl-4-phenylpyridinium (MPP(+)) toxicity in dopaminergic neurons. Brain Res 1197:143-151,2008.
  7. Iwanaka Y,Okada K,Tanaka Y,Takechi U,Tsuji S:Unilateral upper cervical cord infarct restricted to the right funiculus cuneatus.Intern Med 47:479-479,2008.
  8. Hashimoto T., Matsubara S., Mochizuki Y, Tsuji S, Mizutani T, Oyanagi K. Forme fruste or incipient form of widespread-type ALS, or MND with PNLA? An autopsy case report.Neuropathology 28:309-316, 2008.

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  1. Hasegawa M, Arai T, Akiyama H, Nonaka T, Mori H, Akiyama H, Nonaka T, Mori H, Hashimoto T, Yamazaki M, Oyanagi K. TDP-43 is deposited in the Guam parkinsonism-dementia complex brains. Brain 130:1386-1394, 2007.
  2. Katsuno M, Banno H, Suzuki K, Takeuchi Y, Minamiyama M, Waza M, Adachi H, Tanaka F, Sobue G. Therapeutic strategies for spinal and bulbar muscular atrophy (SBMA). Drug Future 32: 907-917, 2007.
  3. Tanaka KI, Namba T, Arai Y, Fujimoto M, Adachi H, Sobue G, Takeuchi K, Nakai A, Mizushima T. Genetic evidence for a protective role for heat shock factor 1 and heat shock protein 70 against colitis. J Biol Chem 282: 23240-23252, 2007.
  4. Jiang YM, Yamamoto M, Tanaka F, Ishigaki S, Katsuno M, Adachi H, Niwa J, Doyu M, Yoshida M, Hashizume Y, Sobue G. Gene Expressions Specifically Detected in Motor Neurons (Dynactin 1, Early Growth Response 3, Acetyl-CoA Transporter, Death Receptor 5, and Cyclin C) Differentially Correlate to Pathologic Markers in Sporadic Amyotrophic Lateral Sclerosis. J Neuropathol Exp Neurol 66: 617-627, 2007.
  5. Adachi H, Waza M, Tokui K, Katsuno M, Minamiyama M, Tanaka F, Doyu M, Sobue G.CHIP overexpression reduces the mutant AR protein and ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model. J Neurosci 27: 5115-5126, 2007.
  6. Adachi H, Waza M, Katsuno M, Tanaka F, Doyu M, Sobue G. Pathogenesis and molecular targeted therapy of spinal and bulbar muscular atrophy. Neuropathol Appl Neurobiol 33:135-151, 2007.
  7. Hashimoto T,Matsubara S,Mochizuki Y,Tsuji S,Mizutani T,Oyanagi K:Forme fruste or incipient form of widespread-type amyotrophic lateral sclerosis, or motor neuron disease with pallido-nigro-luysian atrophy?.Neuropathology  28:309-316,2007
  8. Ohnari K,Yamano M,Uozumi T,Hashimoto T,Tsuji S,Nakagawa M:An adult form of Alexander disease: a novel mutation in glial fibrillary acidic protein.J Neurol 254:1390-1394,2007.
  9. Mera T,Fujihara H,Saito J,Kawasaki M,Hashimoto H,Saito T,Shibata M,Onaka T,Tanaka Y,Oka T,Tsuji S,Ueta, Y:Downregulation of prolactin-releasing peptide gene expression in the hypothalamus and brainstem of diabetic rats.Peptides 28:1596-1604,2007.
  10. Okada K,Tsuji S,Tanaka K:Intermittent Intravenous Immunoglobulin Successfully Prevents Relapses of Neuromyelitis Optica.Intern Med 46:1671-1672,2007.
  11. Hayashida S,Oka T,Tsuji S:Cilnidipine lowered psychological stress-induced increase in blood pressure in a hypertensive man: a case report.Biophychosoc Med 1:1-5,2007.
  12. Tanaka Y,Nakajima M,Hirano T,Uchino M:Cardioembolic stroke followed by isolated celiac artery thromboembolism.Intern Med 46:1463-1466,2007.
  13. Oka T,Oka K:Age and gender differences of psychogenic fever: a review of the Japanese literature.Biopsychosoc Med 1:1-5,2007.
  14. Ishikawa S,Matsunaga K,Nakanishi R,Kawahira K,Murayama N,Tsuji S,Ying-Zu Huang,John C. Rothwell:Effect of theta burst stimulation over the human sensorimotor cortex on motor and somatosensory evoked potentials.Clin Neurophysiol 118:1033-1043,2007.
  15. Umehara F,Nose H,Saito M,Fukuda M,Ogino M,Toyota T,Yuhi T,Arimura K,Osame M:Abnormalities of spinal magnetic resonance images implicate clinical variability in human T-cell lymphotropic virus type I-associated myelopathy.J Neurovirol 13:260-267,2007.

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  1. Hashimoto T, Nishi K, Nagasao J, Tsuji S, Oyanagi K. Magnesium (Mg) effect for MPP+ toxicity on cultured nigral neurons: Aiming to treat Parkinson disease.Brain Pathology 16:S56, 2006.
  2. Katsuno M, Adachi H, Minamiyama M, Waza M, Tokui K, Banno H, Suzuki K, Onoda Y,Tanaka F, Doyu M, Sobue G. Reversible disruption of dynactin 1-mediated retrograde axonal transport in polyglutamine-induced motor neuron degeneration. J Neurosci 26: 12106-12117, 2006.
  3. Waza M, Adachi H, Katsuno M, Minamiyama M, Tanaka F, Sobue G. Alleviating Neurodegeneration by an Anticancer Agent: An Hsp90 Inhibitor (17-AAG). Ann N Y Acad Sci 1086:21-34, 2006.
  4. Matsushita T, Adachi H, Watanabe H, Shimoyama Y, Adachi T, Sobue G, Ito M, Kojima T, Saito H, Naoe T. Classic polyarteritis nodosa presenting rare clinical manifestations in a patient with hemophilia a. Int J Hematol 83: 420-425, 2006.
  5. Waza M, Adachi H, Katsuno M, Minamiyama M, Tanaka F, Doyu M, Sobue G. Modulation of Hsp90 function in neurodegenerative disorders: a molecular-targeted therapy against disease-causing protein. J Mol Med 84: 635-646, 2006.
  6. Katsuno M, Adachi H, Waza M, Banno H, Suzuki K, Tanaka F, Doyu M, Sobue G. Pathogenesis, animal models and therapeutics in Spinal and bulbar muscular atrophy (SBMA). Exp Neurol 200:8-18, 2006.
  7. Banno H, Adachi H, Katsuno M, Suzuki K, Atsuta N, Watanabe H, Tanaka F, Doyu M, Sobue G. Mutant androgen receptor accumulation in SBMA scrotal skin: A pathogenic marker. Ann Neurol 59: 520-526, 2006.
  8. Ando T,Komaki G,Naruo T,Okabe K,Takii M,Kawai K,Konjiki F,Takei M,Oka T,Takeuchi K,Masuda A,Ozaki N,Suematsu H,Denda K,Kurokawa, N,Itakura K,Yamaguchi C,Kono M,Suzuki T,Nakai Y: Possible Role of Preproghrelin Gene Polymorphisms in Susceptibility to Bulimia Nervosa.Am J Med Genet B Neuropsychiatr Genet 141B:929-934,2006.
  9. Ohnari K,Matsunaga K,Uozumi T,Tamagawa A,Hashimoto T,Tsuji S:Unilateral Positive-Negative Myclonus in Creutzfeldt-Jakob Disease.Mov Disord 21: 1963-1966,2006.
  10. Mera T,Fujihara H,Kawasaki M,Hashimoto H,Saito T,Shibata,J,Saito J,Oka T,Tsuji S,Onaka T.,Ueta Y:Prolactin-releasing peptide is a potent mediator of stress responses in the brain through the hypothalamic paraventricular nucleus.Neuroscience 141: 1069-1086, 2006.
  11. Okada K,Yamashita U,Tsuji S: Cyclooxygenase System Contributes to the Maintenance of Post Convulsive Period of Epileptic Phenomena in the Genetically Epileptic El Mice.J UOEH 28: 265-275,2006.
  12. Okada K,Yamashita U,Tsuji S: Ameliorative effect of pioglitazone on seizure responses in genetically epilepsy-susceptible EL mice.Brain Res 1102: 175-178, 2006.
  13. Eiichirou Urasaki,Maeda R,Akamatsu N,Yokota A: Origine and characteristics of high-frequency(>500Hz)SEP components directly recorded from the cervical cord, thalamus, and cerebral cortex.Suppl Clin Neurophysiol 59: 159-165, 2006.
  14. Oka T:Role of Kampo in Treating Stress-related Diseases and Pre-morbid Health Problems.Evolv Kampo 2: 54-57, 2006.
  15. Tsuji S,Uozumi T,Akamatsu N,Tamagawa A,Matsunaga K,Ishiguchi H,Hashimoto T,Kojima Y:Mechanism of voluntary and involuntary movements in humans.Suppl Clin Neurophysio 59: 135-142,2006.
  16. Uozumi T,Tamagawa A,Hashimoto T,Tsuji S: High-frequency oscillations in the human motor system .Suppl Clin Neurophysio 59:143-147,2006.
  17. Komine N,Ohnari K,Murakami M,Kawata K,Suzuka K,Nakamata T: Ischemic Ocular Motor Nerve Palsy in Three Patients with End-Stage Renal Disease.J UOEH 28:57-64,2006.
  18. Okada K,Fujiwara H,Tsuji S: X-linked Charcot-Marie-Tooth Disease with Transient Splenium Lesion on MRI.Intern Med 45: 33-34, 2006.

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  1. Katsuno M, Sang C, Adachi H, Minamiyama M, Waza M, Tanaka F, Doyu M, Sobue G. Pharmacological induction of heat shock proteins alleviates polyglutamine-mediated motor neuron disease. Proc Natl Acad Sci U S A 102:16801-16806, 2005.
  2. Waza M*,Adachi H*, Katsuno M, Minamiyama M, Sang C, Tanaka F, Inukai A, Doyu M, Sobue G.17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration. Nat Med 11: 1088-1095, 2005.(* Evenly contributed to the study)
  3. Jiang Y, Yamamoto M, Kobayashi Y, Yoshihara T, Liang Y, Terao S, Takeuchi H, Ishigaki S, Katsuno M, Adachi H, Niwa J, Tanaka F, Doyu M, Sobue G. Gene expression profiles of spinal motor neurons in sporadic amyotrophic lateral sclerosis. Ann Neurol 57:236-251, 2005.
  4. Adachi H, Katsuno M, Minamiyama M, Waza M, Sang C, Nakagomi Y, Kobayashi Y, Tanaka F, Doyu M, Inukai A, Yoshida M, Hashizume Y and Sobue G. Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients. Brain 128: 659-670, 2005.
  5. Uozumi T,Tamagawa A,Hashimoto T,Tsuji S: Rhythmic EMG and EEG Activity during Voluntary Movement in Posthypoxic Cortical Action Myoclonus.J UOEH 27:227-236,2005.
  6. Okada K,Kuroda E,Yoshida Y,Yamashita U,Suzumura A,Tsuji S: Effects of interferon-β on the cytokine production of astrocytes.J Neuroimmunol 159: 48-54,2005.
  7. Okada K,Yamashita U,Tsuji S: Modulation of Na+-dependent glutamate transporter of murine astrocytes by inflammatory mediators.J UOEH 27: 161-170,2005.
  8. Matsunaga K,Maruyama A,Fujiwara T,Nakanishi R,Tsuji S,John C. Rothwell:Increased corticospinal excitability after 5Hz rTMS over the human supplementary motor area.J Physiol 562:295-306,2005.

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  1. Minamiyama M, Katsuno M, Adachi H, Waza M, Sang C, Kobayashi Y, Tanaka F, Doyu M, Inukai A, Sobue G. Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Hum Mol Genet 13:1183-1192, 2004.
  2. Katsuno M, Adachi H, Sobue G: Sweet relief for Huntington disease. Nat Med 10:123-124, 2004.
  3. Katsuno M, Adachi H, Tanaka F, Sobue G: Spinal and bulbar muscular atrophy: ligand-dependent pathogenesis and therapeutic perspectives. J Mol Med 82: 298-307, 2004.
  4. Ando T,Kodama N,Ishikawa T,Naruo T,Tachikawa N,Nozaki T,Okabe K,Takeuchi K,Masuda A,Kawamura N,Komaki G: Uncoupling Protein-2/uncoupling protein-3 gene polymorphism is not associated with anorexia nervosa.Psychiatr Genet 14:215-218,2004.
  5. 100.Oka, T:Prostaglandin E2 as a mediator of fever: the role of prostaglandin E(EP) receptors.Front Biosci 9:3046-3057,2004.
  6. Uozumi T,Tamagawa A,Hashimoto T,Tsuji S:Motor hand representation in cortical ara44.Neurology 62:757-761,2004.
  7. Kelly JF,Elias CF,Lee CE,Ahima RS,Seeley RJ,Bjorbak C,Oka T,Saper CB,Flier JS,Elmquist JK: Ciliary Neurotrophic Factor and Leptin Induce Distinct Patterns of Immediate Early Gene Expression in the Brain.Diabetes 53:911-920,2004.
  8. Rumi Kumagai-Eto,Kaseda Y,Tobimatsu S,Uozumi T,Tsuji S,Nakamura S: Subclinical cranial nerve involvement in hereditary motor and sensory neuropathy : a combined conduction study with electrical and magnetic stimulation.Clin Neurophysiol 115:1689-1696,2004.
  9. Matsunaga K,Michael A. Nitsche,Tsuji S,John C. Rothwell: Effect of transcranial DC sensorimotor cortex stimulation on somatosensory evoked potentials in humans.Clin Neurophysiol 115:456-460,2004.
  10. Ishihara K, Yamagishi N, Saito Y, Adachi H, Kobayashi Y, Sobue G, Ohtsuka K and Hatayama T: Hsp105 suppresses the aggregation of truncated androgen receptor with expanded CAG repeats and cell toxicity. J Biol Chem 278: 25143-25150, 2003.
  11. Katsuno M, Adachi H, Doyu M, Minamiyama M, Sang C, Kobayashi Y, Inukai A and Sobue G: Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy. Nat Med 9: 768-773, 2003.
  12. Katsuno M, Adachi H, Sobue G. Transgenic mouse models of spinal and bulbar muscular atrophy. Cytogenet Genome Res 100: 243-251, 2003.
  13. Adachi H, Katsuno M, Minamiyama M, Sang C, Pagoulatos G, Angelidis C, Kusakabe M, Yoshiki A, Kobayashi Y, Doyu M and Sobue G. Heat shock protein 70 chaperone over-expression ameliorates phenotypes of the SBMA transgenic mouse model by reducing nuclear-localized mutant AR protein. J Neurosci 23: 2203-2211, 2003.
  14. Oka T,Ok, K,Kobayashi T,Sugimoto Y,Ichikawa A,Ushikubi F,Narumiya S,C B Saper:Characteristics of thermoregulatory and febrile responses in mice deficient in prostaglandin EP1 and EP3 receptors.J Physiol 551:945-954, 2003.
  15. Tsuji S,Akamatsu N: Does transcranial magnetics stimulation improve the motor symptoms of Parkinson disease?.J Neurol 25:47-50,2003.
  16. Oka T,Oka K,Clifford B Saper:Contrasting effects of E type prostaglandin (EP) receptor agonists on core body temperature in rats.:Brain Res 968:256-262,2003.
  17. Sang C, Kobayashi Y, Du J, Katsumo M, Adachi H, Doyu M, Sobue G. c-Jun N-terminal kinase pathway mediates Lactacystin-induced cell death in a neuronal differentiated Neuro2a cell line. Brain Res Mol Brain Res 108: 7-17, 2002
  18. Katsuno M*, Adachi H*, Kume A, Li M, Nakagomi Y, Niwa H, Sang C, Kobayashi Y, Doyu M and Sobue G: Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Neuron 35: 843-854, 2002.(* Evenly contributed to the study)
  19. Okada K,Matsunaga K,Yuhi T,Kuroda E,Yamashita U,Tsuji S:The long-term high-frequency repetitive transcranial magnetic stimulation does not induce mRNA expression of inflammatory mediators in the rat central nervous system.Brain Res 957:37-41,2002.
  20. Kojima Y,Hashiguchi H,Hashimoto T,Tsuji S,Shoji H,Kazuyama Y: Recurrent Herpes Simplex Virus Type 2 Meningitis:A Case Report of Mollaret's Meningitis.Jpn J Infect Dis 55:85-88,2002.
  21. Matsunaga,K,Akamatsu N,Uozumi T,Urasaki E,Tsuji S:Early and late inhibition in the human motor cortex studied by paired stimulation through subdural electrodes.Clin Neurophysiol 113:1099-1109,2002.
  22. Uozumi T,Matsunaga K,Tsuji S:Ipsilateral motor-evoked potentials in patients with congenital and acquired mirror movements.Int Congr Ser 1232:915-921,2002.
  23. Adachi H, Kume A, Li M, Nakagomi Y, Niwa H, Do J, Sang C, Kobayashi Y, Doyu M, Sobue G: Transgenic mice with an expanded CAG repeat controlled by the human AR promoter show polyglutamine nuclear inclusions and neuronal dysfunction without neuronal cell death. Hum Mol Genet 10: 1039-1048, 2001
  24. Kojima Y,Uozumi T,Akamatsu N,Matsunaga K,Urasaki E,Tsuji S: Somatosensory evoked high frequency oscillations recorded from subdural electrodes.Clin Neurophysiol 112:2261-2264,2001.
  25. Akamatsu N,Fueta Y,Endo Y,Matsunaga K,Uozumi T,Tsuji S: Decreased susceptibility to pentylenetetrazol-induced seizures after low-frequency transcranial magnetic stimulation in rats.Neurosci Lett 14:153-156,2001.
  26. Okada K,Sugiura T,Kuroda E,Tsuji S,Yamashita U:Phenytoin promotes Th2type immune response in mice.Clin Exp Immunol 124:406-413,2001.
  27. Im HJ,Smirnov D,Yuhi T,Raghavan S,Olsson JE,Muscat GE,Koopman P,Loh HH: Transcripional modulation of mouse mu-opioid receptor distal promoter activity by Sox 18.Mol Pharmacol 59:1486-1496,2001.
  28. Okada K,Yuhi T,Tsuji S,Yamashita U: Cyclooxygenase-2 expression in the hippocampus of genetically epilepsy susceptible El mice was increased after seizure. Brain Res 894:332-335,2001.
  29. Matsunaga K,Uozumi T,Hashimoto T,Tsuji S: Cerebellar stimulation in acute cerebellar ataxia.Clin Neurophysiol 112:619-622,2001.
  30. Matsunaga K,Uozumi T,L. Qingrui,Hashimoto T,Tsuji S:Amantadine-induced cortical myoclonus.Neurology 56:279-280,2001.
  31. Hashimoto T,Uozumi T,Tsuji S: Paraspinal motor evoked potentials by magnetic stimulation of the motor cortex.Neurology 55:885-888,2000.
  32. Ohnishi A,Yamamoto T,Ikeda M: Small axons relative to number of myelin lamellae in Charcot-Marie-Tooth disease 1A with peripheral myelin protein 22 gene duplication.J UOEH 22:107-117,2000.
  33. Fukuda M,Morimoto T,Ishida Y,Suzuki Y,Murakami Y,Kida K,Ohnishi A: Improvement of peripheral neuropathy with oral prednisolone in Chediak-Higashi syndrome.Eur J Pediatr 159:300-301,2000.
  34. Ohnishi A,Yamamoto T,Izawa K,Yamamori S,Takahashi K,Mega H,Jinnai K:Dejerine-Sottas disease with a novel de novo dominant mutation, Ser 149 Arg, of the peripheral myelin protein 22.Acta Neuropathol 99:327-330,2000.
  35. Okada K,Yoshida Y,Sugiura T,Tanaka H,Tsuji S,Yamashita H,Yamashita U:Reduced interleukin-1β responsiveness in immune system and central nervous system of inbred polydipsic(STR/N)mice.Life Sci 66:1461-1470, 2000.
  36. Matsunaga K,Uozumi T,Akamatsu N,Nagashio Y,Liu Qingrui,Hashimoto T,Tsuji S:Negative myoclonus in Creutzfeldt-Jakob disease.Clin Neurophysiol 111:471-476,2000.
  37. Ben Othmane K,Johnson E,Menold M,Graham F.C,Ben Hamida M,Hasegawa O,Rogala A.D,Ohnishi, A,Pericak-Vance M,Hentati F,Vance,J.M: Identification of a new locus for autosomal recessive Charcot-Marie-Tooth disease with focally folded myelin on chromosome 11p 15.Genomics 62:344-349,1999.
  38. Ohnishi A,Yamamoto T,Yamamori S,Sudo K,Fukushima Y,Ikeda M: Myelinated fibers in Charcot-Marie-Tooth disease type 1B with Arg98His mutation of Po protein.J Neurol Sci 171:97-109,1999.
  39. Ohnishi A,Yamamoto T,Izawa K,Yamamori S,Takahashi K,Mega, H,Jinnai K:Dejerine-Sottas disease with a novel de novo dominant mutation, Ser 149 Arg, of the peripheral myelin protein 22.Acta Neuropathol 99:327-330,1999.
  40. Izumi Horiuch,Yuji Kawano,Kenji Yamasaki,Hidenori Matsuo,Motozumi Minohara,Tatsufumi Nakamura,Hashiguchi H,Takeshi Yamada: Heightened IgE response to mite antigens in inflammatory neuropathies.J Neuro Sci 166:77-80,1999.
  41. Yanagita T,Kobayashi H,Yamamoto R,Takami Y,Yokoo H,由比 友顕,Nakayama T,Wada A:Protein kinase C and the opposite regulation of sodium channel alpha-and betal-subunit mRNA levels in adrenal chromaffin cells.J Neurochem 73:1749-1757,1999.
  42. Jun-ichi Satoh,Yamamoto T,Yasuo Kuroda:Vasculitis confined to the peripheral nerve and skin:a varient of non-systemic vasculitic neuropathy.Eur J Neurol :1999.
  43. Kakimi S,Goto N,Otsuka N,Ohnishi A:Neuropathologic abnormalities of the brain in the Wistar ataxic rat.Neuropathology :398-401,1998.
  44. Matsunaga K,Uozumi T,Tsuji S,Murai, Y:Age-dependent changes in physiological threshold asymmetries for the motor evoked potential and silent period following transcranial magnetic stimulation.Electroencephalogr Clin Neurophysiol 109:502-507,1998.
  45. Matsunaga K,Uozumi T,Tsuji S,Murai Y:Sympathetic skin responses recorded from non-palmar and non-plantar skin sites: their role in the evaluation of thermal sweating.Electroencephalogr Clin Neurophysiol 108:482-489,1998.
  46. Miyazoe S,Matsuo H,Ohnishi A,Tajima F,Fujishita S,Ichinose K,Shibuya N:Acquired idiopathic generalized anhidrosis with isolated sudomotor neuropathy.Ann Neurol 44:378-381,1998.
  47. Ohnishi A,Yamamoto T,Murai Y,Ando Y,Ando M,Hoshii Y,Ikeda M:Denervation of eccrine glands in patients with familial amyloidotic polyneuropathy type. Neurology 51:714-721,1998.
  48. Yabe I,Sasaki H,Matsuura T,Takada A,Wakisaka A,Suzuki Y,Fukazawa T,Hamada T,Oda T,Ohnishi A,Tashiro K: SCA 6 mutation analysis in a large cohort of the Japanese patients with late-onset pure cerebellar ataxia .J Neurol Sci 156:89-95,1998.
  49. Yamamoto M,Keller MP,Yasuda T,Hayasaka K,Ohnishi A,Yoshikawa H,Yanagihara T,Mitsuma T,Chance PF,Sobue G: Clustering of CMT1A duplication breakpoints in a 700 bp interval of the CMT1A-REP repeat .Hum Mutat 11:109-113,1998.
  50. Yoshimura T,Satake M,Ohnishi A,Tsutsumi Y,Fujikura Y:Mutations of connexin 32 in Charcot-Marie-Tooth type communication but not cell proliferation and myelin-specific gene expression.J Neurosci. Res 51:154-161,1998.
  51. Yoshimura T,Satake M,Ohnishi A,Tsutsumi Y,Fujikura Y:Mutations of connexin 32 in Charcot-Marie-Tooth type communication but not cell proliferation and myelin-specific gene expression.J Neurosci. Res 51:154-161,1998.
  52. Han M.F,Her Q.Y,Ohnishi A,Yamamoto T,Hashiguchi H,Murai Y: Nuclear pore changes and absence of apotosis in lumbar dorsal root ganglion neurons of doxorubicin-intoxicated rats.J UOEH 20:115-125,1998.
  53. Sashihara S,Tsuji S,Matsui T:Oncogenes and signal transduction pathways involved in the regulation of Na+channel expression.Crit Rev Oncog 9:19-34,1998.
  54. Komiyama A.K,Ohnishi A,Izawa K,Yamamori S,Ohashi H,Hasegawa O: De novo mutation (Arg98→Cys) of the myelin Po gene and uncompaction of the major dense line of the myelin sheath in a severe variant of Charcot-Marie-Tooth disease type 1B.J Neuro Sci 149:103-109,1997.
  55. Shimizu T,Yamashita Y,Satoi M.,Togo A,Wada N,Matsuishi T,Ohnishi A,Kato H:Heat stroke-like episode in a child caused by zonisamide.Brain Dev 19: 366-368,1997.
  56. Ikegami T,Ikeda H,Chance P.F,Kiyosawa,H,Yamamoto M,Sobue G,Ohnishi A,Tachi N,Hayasaka K:Facilitated Diagnosis of CMT1A Duplication in Chromosome 17p11.2-12 :Analysis With a CMT1A-REP Repeat Probe and Photostimulated Luminescence Imaging.Hum Mutat 9:563-566,1997.
  57. Nagamitsu S,Matsuishi T,Ohnishi,A,Kato H:Decreased CSF levels of substance P in patients with congenital sensory neuropathy with anhidrosis.Neurology 48:1133-1133,1997.
  58. Nakano S,Imamura S,Tokunaga K,Tsuji S,et al.:Evoked Potentials in Patients with Chronic Respiratory Insufficiency.Intern Med 36:270-275,1997.
  59. Yamamoto R,Wada A,Asada Y,Yanagita T,Yuhi T,Niina H,Sumiyoshi A,Kobayashi H,Lee TJ: Nitric oxide-dependent and -independent norepinephrine release in rat mesenteric arteries.Am J Physiol 272:H207-H210,1997.
  60. Satake M,Yoshimura T,Ohnishi A,Kobayashi T:Connexin32 gene expression in rat sciatic nerves and cultured schwann cells.Dev Neurosci 19:189-195, 1997.
  61. Yamamoto M,Yasuda T,Hayasaka,K,Ohnishi A,et al.:Locations of crossover breakpoints within the CMT1A-REP repeat in Japanese patients with CMT1A and HNPP.Hum Genet 99:151-154,1997.
  62. Ono T,Fueta Y,Janjua N,Ohmori O,Ohno K:Granule cell disinhibition in dentate gyrus of genetically seizure susceptible El mice.Brain Res 745:165-172,1997.
  63. Satoh J,Tokumoto H,Kurohara K,Yukitake M,Matsui M,Kuroda,Y,Yamamoto T,et al.:Adult-onset Krabbe disease with homozygous T1853C mutation in the galactocerebrosidase gene.Neurology 49:1392-1399,1997.
  64. Yoshimura T,Ohnishi H,Yamamoto T,Kitani M:Two novel mutations (C53S, S26L) in the connexin32 of Charcot-Marie-Tooth disease type X families. Hum Mutat 8:270-272,1996.
  65. Yamamoto K,Ohnishi A:Ascending myelinated axons of primary sensory neurons of the sciatic nerve in the posterior column of the rat spinal cord.Acta Neuropathol 92:27-34,1996.
  66. Hosokawa S,Tsuji S,Uozumi T,Matsunaga,K:Ipsilateral hemiplegia caused by right internal capsule and thalamic hemorrhage: Demonstration of predominant ipsilateral innervation of motor and sensory systems by MRI, MEP, and SEP.Neurology 46:1146-1149,1996.
  67. Niina H,Kobayashi H,Yamamoto R,Yuhi T,Yanagita T,Wada A:Receptors for artial natriuretic peptide in adrenal chromaffin cells.Biochem Pharmacol 51:855-858,1996.
  68. Yamamoto R,Yanagita, T,Kobayashi H,Yuhi T,Yokoo H,Wada A:Up-regulation of functional voltage-dependent sodium channels by insulin in cultured bovine adrenal chromaffin cells. J Neurochem 67:1401-1408,1996.
  69. Yanagita T,Wada,A,Yamamoto,R,Kobayashi H,Yuhi T,Urabe M,Niina:Protein kinase C-mediated down-regulation of voltage-dependent sodium channels in adrenal chromaffin cells.J Neurochem 66:1249-1253,1996.
  70. Yuhi T,Wada A,Kobayashi H,Yamamoto R,Yanagita T,Niina H:Up-regulation of functional voltage dependent sodium channels by cyclic AMP-dependent protein kinase in adrenal medulla.Brain Res 709:37-43,1996.
  71. Nakano S,Tsuji S,Matsunaga K,Murai Y:Effect of sleep stage on somatosensory evoked potentials by median nerve stimulation.Electroencephalogr Clin. Neurophysiol. 96:385-389,1995.
  72. Ohnishi A,Sonobe M:Asian hereditary neuropathy patients with peripheral myelin protein-22 gene aneuploidy. Am J Med Genet 59:51-58,1995.
  73. Deng H.X,Tainer J.A,Mitsumoto H,Ohnishi A,He X,Hung W.Y,Zhao Y,Juneja T,Hentati A,Siddique T:Two novel SOD1 mutations in patients with familial amyotrophic lateral sclerosis.Hum Mol Genet 4:1113-1116,1995.
  74. Jiang X.M,Ohnishi A,Yamamoto T,Murai Y,Awaya A,Ikeda M:The effect of MS-818, a pyrimidine compound, on the regeneration of peripheral nerve fibers of mice after a crush injury.Acta Neuropathol 90:130-134,1995.
  75. Wada A,Urabe M,Yuhi T,Yamamoto R,Yanagita T,Niina H,Kobayashi H:Large-and small-conductance Ca(2+)-activate K+ channels: their role in the nicotinic receptor-mediated catecholamine secretion in bovine adrenal medulla.Naunyn Schmiedebergs Arch Pharmacol 352:545-549,1995.
  76. Matsunaga K,Uozumi T,Tsuji S,Murai Y:Sympathetic skin responses evoked by magnetic stimulation of the neck.J Neuro Sci 128: 188-194,1995.
  77. Zhao J.X,Ohnishi A,Yamamoto T,Hayashi H:Greater number of microtubules per axon of unmyelinated fibers of mutant quails deficient in neurofilaments : possible compensation for the absence of neurofilaments.Acta Neuropathol 87:332-336,1994.
  78. Zhao J.X,Ohnishi A,Yamamoto T,Hojo T:Smaller axon and unaltered numbers of microtubules per axon in relation to number of myelin lamellae of myelinated fibers in the mutant quail deficient in neurofilaments.Acta Neuropathol 89:305-312,1994.
  79. Yamamoto R,Wada A,Asada Y,Uozumi T,Yanagita T,Niina H,Sumiyoshi A:Functional relation between nitric oxide and noradrenaline for the modulation of vascular tone in rat mesenteric vasculature.Naunyn Schmiedebergs Arch Pharmacol 349:362-366,1994.
  80. Hayasaka K,Ohnishi A,Takada G,Fukushima Y,Murai Y:Mutation of the myelin Po gene in Charcot-Marie-Tooth neuropathy Type I.Biochem Biophys Res Commun 194:1317-1322,1993.
  81. Shiomi K,Nakazato M,Matsukura S,Ohnishi A,Hatanaka H,Tsuji S,Murai Y,Kojima M,Kangawa K,Matsuo H:A basic transthyretin variant (Glu61→Lys) causes familial amyloidotic polyneuropathy: Protein and DNA sequencing and PCR-induced mutation restriction analysis.Biochem Biophys Res Commun 194:1090-1096,199308.
  82. Oishi T,Hasegawa E,Murai Y:Sulfhydryl drugs reduce neurotoxicity・6 of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) in the mouse.J Neural Transm Park Dis Dement Sect 6:45-52,1993.
  83. Uozumi T,Nakano S,Matsunaga K,Tsuji S,Murai Y:Sudomotor Potential Evoked by Magnetic Stimulation of the Neck.Neurology 43:1397-1400,1993.
  84. Zhao J.X,Ohnishi A,Yamamoto T,Hayashi H:Smaller number of large myelinated fibers and focal myelin thickening in mutant quails deficient in neurofilaments.Acta Neuropathol 86:242-248,1993.
  85. Madrid R.G,Ohnishi A,Hachisuka K,Murai Y:Axonal sprouting of motor nerve in acrylamide - intoxicated rats with progressive weakness.Environ Res 60:233-241,1993.
  86. Ohnishi A,Murai Y:Polyneuropathy due to ethylene oxide, propylene oxide, and butylene oxide.Environ Res 60: 242-247,1993.
  87. Lin SH,Ohnishi A,Jiang X-M,Yamamoto T:Clinical and pathological studies on peripheral neuropathy of six chinese patients with lung cancer: Morphometric evaluation of the sural nerve on biopsy. J UOEH 15:13-20,199
  88. Yoshinaga T,Nakazato M,Ikeda S,Ohnishi A:Homozygosity for the transthyretin-Met 30 gene in three Japanese sibling with type I familial amyloidotic polyneuropathy.Neurology 42:2045-2047,1992.
  89. Nagata H,Ohkoshi N,Kanazawa ,Oka N,Ohnishi A:Rapid axonal transport velocity is reduced in experimental ethylene oxide neuropathy.Mol Chem Neuropathol 17:209-217,1992.
  90. Sasaki K,Tachi K,Shinoda M,Satoh N,Minami R,Ohnishi A:Demyelinating peripheral neuropathy in Cockayne syndrome: a histologic and morphometric study. Brain Dev 14:114-117,1992.
  91. Uozumi T,Ito Y,Tsuji S,Murai Y:Inhibitory period following motor evoked potentials evoked by magnetic cortical stimulation.Electroencephalogr Clin Neurophysiol 85:273-279,1992.
  92. Ohnishi A:Polyneuropathy due to ethylene oxide, propylene oxide and butylene oxide.Neuropathology 4:714-717,1991.
  93. Uozumi T,Tsuji S:Motor potentials evoked by magnetic stimulation of the motor cortex in normal subjects and patients with motor disorders.Electroencephalogr Clin Neurophysiol 81:251-256,1991.
  94. Mita T,Sashihara S,Aramaki I,Fueta Y,Hirano H:Unusual biochemical development of genetically seizuresusceptible E1 mice.Dev Brain Res 64:27-35,1991.
  95. Tsuji S,Murai Y:Cortical somatosensory potentials evoked by magnetic stimulation:effect of body height, age, and stimulus intensity. Electroencephalogr Clin Neurophysiol 80:32-38,1991.
  96. Tsuji S,Uozumi T,Itoh Y,Ohnishi A,Murai Y:Serial somatosensory evoked potentials in a patient with familial hypobetalipoproteinemia, and vitamine E deficiency.Jpn J Med 29:628-632,1990.
  97. Tsuji S,Murai Y:Cortical somatosensory evoked potentials by magnetic stimulation.Brain Topogr 3:227-228,1990.
  98. Hasegawa E,Takeshige K,Oishi T,Murai Y,Minakami S:1-Methy1-4-Phenylpyridinium (MPP+) induces NADH-dependent superoxide formation and enhances NADH-dependent lipid peroxidation in bovine heart submitochondrial particles.Biochem Biophys Res commun 70:1049-1055.1990.
  99. Tsuji S,Uozumi T,Nakano S,Ohnishi A,Murai Y:Evoked potential studies in mitochondrial encephalomyopathy.Arch Neurol 47:465-467,1990.
  100. Kanda F,Uchida T,Jinnai K,Tada K,Shiozawa S,Fujita T,Ohnishi A: Acute autonomic and sensory neuropathy : a case report.J Neurol 237:42-44,1990.
  101. Ohnishi A,Murai Y,Ikeda M,Fujita T,Furuya H,et al.:Autosomal recessive motor and sensory neuropathy with excessive myelin outfolding.Muscle Nerve 12:568-575,1989.

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