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2002 Fiscal Year Final Research Report Summary

STUDIES ON MOLECULAR AND CLINICAL PATHOMECHANISM OF SPINOCEREBELLAR DEGENERATION

Research Project

Project/Area Number 12307013
Research Category

Grant-in-Aid for Scientific Research (A)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionTokyo Medical and Dental University

Principal Investigator

MIZUSAWA Hidehiro  TOKYO MEDICAL AND DENTAL UNIVERSITY, DEPARTMENT OF NEUROLOGY AND NEUROLOGICAL SCIENCE, PROFESSOR, 大学院・医歯学総合研究科, 教授 (30144091)

Co-Investigator(Kenkyū-buntansha) KANOUCHI Tadashi  TOKYO MEDICAL AND DENTAL UNIVERSITY, DEPARTMENT OF NEUROLOGY AND NEUROLOGICAL SCIENCE, ASSISTANT LECTURER, 医学部附属病院, 助手 (50345287)
ISHIKAWA Kinya  TOKYO MEDICAL AND DENTAL UNIVERSITY, DEPARTMENT OF NEUROLOGY AND NEUROLOGICAL SCIENCE, ASSISTANT LECTURER, 医学部附属病院, 助手 (30313240)
YOKOTA Takanori  TOKYO MEDICAL AND DENTAL UNIVERSITY, DEPARTMENT OF NEUROLOGY AND NEUROLOGICAL SCIENCE, ASSISTANT PROFESSOR, 医学部附属病院, 講師 (90231688)
Project Period (FY) 2000 – 2002
KeywordsSPINOCEREBELLAR DEGENERATION / CAG REPEAT / SCA6 / CA CHANNEL / PURKINJE CELL / ANIMAL MODEL / CACNA1A / αTTP
Research Abstract

In the research on pathogenesis of spinocerebellar ataxia type 6 (SCA6), we found Purkinje cell specific inclusions and SCA6 gene resulted in Ca channel abnormality in cultured cells. A new gene locus for pure cerebellar ataxia was disclosed and the candidate area was narrowed. Regarding glial cytoplasmic inclusions in multiple system atrophy, we identified the 7 KD core fragment of α-synuclein molecule.

  • Research Products

    (39 results)

All Other

All Publications (39 results)

  • [Publications] Mizusawa, _H, Ishii, T, Bannai, S: "Peroxiredoxin I (macrophage 23 kDa Stress Protein) is highly and widely expressed in the rat nervous system"Neurosci Letters. 283. 57-60 (2000)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Mizusawa, _H, Mitoma, H, Song, SY, Ishida, K, Yamakuni T, Kobayashi, T: "Presynapic impairment of cerebellar inhibitory synapses by an autoantibody to glutamate decarboxylase"J Neurol Sci. 175. 40-44 (2000)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Mizusawa, H, Toru, S, Murakoshi, T, Ishikawa, K, Saegusa, H, Fujigasaki, H, Uchihara, T, Nagayama, S, Osanai, M, Tanabe, T: "Spinocerebellar ataxia type 6 mutation alters P-type calcium channel function. J Biol Chem 275"J Biol Chem. 275. 10893-10898 (2000)

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      「研究成果報告書概要(和文)」より
  • [Publications] Mizusawa, H, Yang, Q, Hashizume, Y, Yoshida, M, Wang, Y, Goto, Y, Mitsuma, N, Ishikawa, K: "Morphological purkinje cell changes in spinocerebellar ataxia type 6"Acta Neuropathol. 100. 371-376 (2000)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Mizusawa, H, Yokota, T, Uchihara, T, Kumagai, J, Shiojiri, T, Pang, JJ, Arita, M, Arai, H, Hayashi, M, Kiyosawa, M, Okeda, R: "Postmortem study of ataxia with retinitis pigmentosa by mutation of the α-tocopherol transfer protein"J Neurol Neurosurg Psychiatry. 68. 521-525 (2000)

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      「研究成果報告書概要(和文)」より
  • [Publications] Mizasawa, H, Kanda, T, Iwasaki, T, Yamawaki, M, Tai, T: "Anti-GM-1 antibody facilitates leakage in an in vitro blood-nerve barrier model"Neurology. 55. 585-587 (2000)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Mizusawa H, Ishi T, Bannai S: "Peroxiredoxin I (macrophage 23 kDa Stress Protein) is highly and widely ecpressed in the rat nervous system"Neurosci Letters. 283. 57-60 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Mitoma H, Song SY, Ishida K, Yamakuni T, Kobayashi T, Mizusawa H: "Presynapic impairment of cerebellar inhibitory synapses by an autoantibody to glutamate decarboxylase"J Neurol Sci. 175. 40-44 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Toru S, Murakoshi T, Ishikawa K, Saegusa H, Fujigasaki H, Uchihara T, Nagayama S, Osanai M, Mizusawa H, Tanabe T: "Spinocerebellar ataxia type 6 mutation alters P-type calcium channel function"J Biol Chem. 275. 10893-10898 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Yang Q, Hashizume Y, Yoshida M, Wang Y, Goto Y, Mitsuma N, Ishikawa K, Mizusawa H: "Morphological purkinje cell changes in spinocerebellar ataxia type 6"Acta Neuropathol. 100. 371-376 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Yokota T, Uchihara T, Kumagai J, Shiojiri T, Pang JJ, Arita M, Arai H, Hayashi M, Kiyosawa M, Okeda R, Mizusawa H: "Postmortem study of ataxia with retinitis pigmentosa by mutation of the α-tocopherol transfer protein"J Neurol Neurosurg Psychiatry. 68. 521-525 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kanda T, Iwasaki T, Yamawaki M, Tai T, Mizasawa H: "Anti-GM-1 antibody facilitates leakage in an in vitro blood-nerve barrier model"Neurology. 55. 585-587 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Nagaoka U, Takashima M, Ishikawa K, Yoshizawa K, Yoshizawa T, Ishikawa M, Yamawaki T, Shoji S, Mizusawa H: "A gene on SCA4 locus causes dominantly inherited pure cerebellar ataxia"Neurology. 54. 1971-1975 (2000)

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  • [Publications] Fujigasaki H, Uchihara T, Koyano S, Iwabuchi K, Yagishita S, Makifuchi T, Nakamura A, Ishida K, Toru S, Hirai S, Ishikawa K, Tanabe T, Mizusawa H: "Ataxin-3 is translocated into the nucleus for the formation of intranuclear inclusions in normal and Machado-Joseph disease brains"Exp Neurol. 165. 248-256 (2000)

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  • [Publications] Kanda T, Iwasaki T, Nakamura S, Kurokawa T, Ikeda K, Mizusawa H: "Self-secretion of fibroblast growth factor-9 supports basal forebrain cholinergic neurons in an autocrine/paracrine manner"Brain Res. 876. 22-30 (2000)

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  • [Publications] Yaginuma M, Ishida K, Uchihara T, Suzuki F, Aoki M, Tanaka T, Murase H, Ikeda K, Mizusawa H: "Paraneoplastic cerebellar ataxia with mild cerebelloolivary degeneration and an anti-neuronal antibody"a clinicopathological study. Neuropathol Appl Neurobiol. 26. 568-572 (2000)

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  • [Publications] Takashima M, Ishikawa K, Nagaoka U, Shoji S, Mizusawa H: "A linkage disequilibrium at the candidate gene locus, for 16q-linked autosomal dominant cerebellar ataxia type III in Japan"J Hum Genet. 46. 167-171 (2001)

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  • [Publications] Ishikawa K, Owada K, Ishida K, Fujigasaki H, Shun LM, Tsunemi T, Ohkoshi N, Toru S, Mizutani T, Hayashi M, Arai N, Hasegawa K, Kawakami T, Kato T, Makifuchi T, Shoji S, Tanabe T, Mizusawa H: "Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells"Neurology. 56. 1753-1756 (2001)

    • Description
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  • [Publications] Miura Y, Misawa N, Maeda N, Inagaki Y, Tanaka Y, Ito M, Kayagaki N, Yamamoto N, Yagita H, Mizusawa H, Koyanagi Y: "Critical contribution of tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) to apoptosis of human CD4+ T cells in HIV-1-infected hu-PBL-NOD-SCID mice"J Exp Med. 193. 651-659 (2001)

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  • [Publications] Zhao F, Kuroiwa T, Miyasaka N, Nagaoka T, Nakane M, Tamura A, Mizusawa H: "Characteristic changes in T2-value, apparent diffusion coefficient, and ultrastructure of substantia nigra evolving exofocal postischemic neuronal death in rats"Brain Res. 895. 238-244 (2001)

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  • [Publications] Takenoshita H, Shizuka IM, Mitoma H, Song S, Harigaya Y, Igeta Y, Yaguchi M, Ishida K, Shoji M, Tanaka M, Mizusawa H, Okamoto K: "Presynaptic inhibition of cerebellar GABAergic transmission by glutamate decarboxylase autoantibodies in progressive cerebellar ataxia"J Neurol Neurosurg. Psychiatry. 70. 386-389 (2001)

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  • [Publications] Zhang L, Miura Y, Ishida R, Saitoh Y, Mizusawa H, Shibuya H: "Differentiating between progressive supranuclear palsy and corticobasal degeneration by brain perfusion SPET"Nucl Med Comm. 22. 767-772 (2001)

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  • [Publications] Ishida K, Takeuchi H, Takahashi R, Yoshimura K, Yamada M, Mizusawa H: "A possible novel isoform of peripheral myelin PO protein : a target antigen recognized by an autoantibody in a patient with malignant lymphoma and peripheral neuropathy"J Neurol Sci. 188. 43-49 (2001)

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  • [Publications] Inaba A, Yokota T, Saito Y, Ichikawa T, Mizusawa H: "Proximal motor conduction evaluated by transcranial magnetic stimulation in acquired inflammatory demyelinating neuropathies"Clinical Neurophysiology. 112. 1936-1945 (2001)

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  • [Publications] Fujigasaki H, Uchihara T, Takahashi J, Matsushita H, Nakamura A, Koyano S, Iwabuchi K, Hirai S, Mizusawa H: "Preferential recruitment of ataxin-3 independent of expanded polyglutamine : an immunohistochemical study on Marinesco bodies"J Neurol Neurosurg Psychiatry. 71. 518-520 (2001)

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  • [Publications] Sodeyama N, Orimo S, Oka T, Nagashima K, Ozawa E, Mizusawa H: "A noble from of reversible subacute spongiform leukoencephalopathy"Eur Neurol. 46. 223-224 (2001)

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  • [Publications] Yokota T, Igarashi K, Uchihara T, Jishage K, Tomita H, Inaba A, Li Y, Arita M, Suzuki H, Mizusawa H, Arai H: "Delayed-onset ataxia in mice lacking α-tocopherol transfer protein : Model for neuronal degeneration caused by chronic oxidative stress"Proc Natl Acad Sci USA. 98. 15185-15190 (2001)

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  • [Publications] Ishibashi S, Yokota T, Inaba A, Yamada M, Mizusawa H: "Mexiletine is effective on segmental hyperhidrosis : report of two cases"J Neurol Neurosurg Psychiatry. 72. 122 (2002)

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  • [Publications] Tsunemi T, Saegusa H, Ishikawa K, Nagayama S, Murakoshi T, Mizusawa H, T. Tanabe: "Novel Cav2.1 splice variants isolated from purkinje cells do not generate P-type Ca2+ current"J. Biological Chemistry. 277(9). 7214-7221 (2002)

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  • [Publications] Inaba A, Yokota T, Otagiri A, Nishimura T, Saito Y, Ichikawa T, Mizusawa H: "Electrophysiological evaluation of conduction in the most proximal motor root segment"Muscle and Nerve. 25. 608-611 (2002)

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  • [Publications] Nishida Y, Yamada M, Hara K, Tsumeni T, Yamawaki M, Shimokawa M, Okeda R, Tsumemi T, Mizusawa H: "Creutzfeldt-Jakob disease after Jannetta's operation with cadaveric dura mater graft : initial manifestations related to the grafted site"J Neurol. 249. 480-483 (2002)

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  • [Publications] Miake H, Mizusawa H, Iwatsubo T, Hasegawa M: "Biochemical characterization of the core structure of α- synuclein filaments"J. Biological Chemistry. 277(21). 19213-19219 (2002)

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  • [Publications] Ohara S, Iwahashi T, Oide T, Hayashi R, Nakajima T, Ishikawa K, Mizusawa H: "Spinocerebellar ataxia type6 with motor neuron loss : A follow-up autopsy report"J. Neurol. 294. 633-635 (2002)

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  • [Publications] Sakamoto M, Uchihara T, Hayashi M, Nakamura A, Kikuchi E, T. Mizutani T, Mizusawa H, Hirai S: "Heterogeneity of nigral and cortical lewy bodies differentiated by amplified triple-labeling for alpha-synuclein, ubiquitin, and thiazin red"Experimental Neurologu. 177. 88-94 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Zhao F, Kuroiwa T, Miyasaka N, Nagaoka T, Nakane M, Tamura A, Mizusawa H: "Ultrastructural and MRI study of the substantia nigra evolving exofocal post-ischemic neuronal desth in the rat"Neuropathology. 22. 91-105 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Miura Y, Misawa N, Inagaki Y, Yamatoto N, Ito M, Yagita H, Okumura K, Mizusawa H, Koyanagi Y: "TNF-related apoptososis-inducing ligand on HIV-1-infected macrophages induces neuronal apoptosis in murine model of HIV encephalopathy"PNAS. in press. (2003)

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  • [Publications] Kubodera T, Yokota T, Ohwada K, Ishikawa K, Miura H, Matsuoka T, Mizusawa H.: "Proteolytic cleavage and cellular toxicity of the human α1A calcium channel in spinocerebellar ataxia type 6 (SCA6)"Neuroscience Letters. in press. (2003)

    • Description
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  • [Publications] Kanda T, Yamawaki M, Mizusawa H: "Sera from Guillain-Barre patients enhance leakage in blood-nerve barrier model"Neurology. 60. 301-306 (2003)

    • Description
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  • [Publications] Mizusawa,H.(Stefan M. Pulst): "Spinocerebellar ataxia type 4 (SCA4), Genetics of movement disorders"Academic press. 71-73 (2002)

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Published: 2004-04-14  

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