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Study for the pathogenesis of neuropathy with Desert hedgehog gene mutation and development for the treatment

Research Project

Project/Area Number 17590884
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionKagoshima University

Principal Investigator

UMEHARA Fujio  Kagoshima University, Graduate School of Medical and Dental Sciences, Assistant Professor, 大学院医歯学総合研究科, 講師 (20271140)

Co-Investigator(Kenkyū-buntansha) TAKASHIMA Hiroshi  Kagoshima University, Graduate School of Medical and Dental Sciences, Research Associate, 大学院医歯学総合研究科, 助手 (80372803)
ARIMURA Kimiyoshi  Kagoshima University, Graduate School of Medical and Dental Sciences, Associate Professor, 大学院医歯学総合研究科, 助教授 (20159510)
OSAME Mitsuhiro  Kagoshima University, Graduate School of Medical and Dental Sciences, Associate Professor, 大学院医歯学総合研究科, 教授 (10041435)
Project Period (FY) 2005 – 2006
Project Status Completed (Fiscal Year 2006)
Budget Amount *help
¥3,500,000 (Direct Cost: ¥3,500,000)
Fiscal Year 2006: ¥1,700,000 (Direct Cost: ¥1,700,000)
Fiscal Year 2005: ¥1,800,000 (Direct Cost: ¥1,800,000)
KeywordsDesert hedgehog / peripheral nerve / diabetes mellitus / Schwann cell / hereditary neuropathy / 再生 / うつ病
Research Abstract

1. Analysis of Desert hedgehog signaling pathway in peripheral nervous system
We found that Dhh receptor patched(ptc)2 was detected in adult sciatic nerves using RT-PCR, however, ptc1 was undetectable under the same experimental condition. We found that the Dhh-Ptc2 signaling pathway may be involved in the maintenance of adult nerves and may be one of the factors that directly or indirectly determines the response of peripheral nerves to injury.
2. Analysis of Dhh function in the central nervous system
We found that Dhh KO male mice exhibited prolonged immobility time compared with wild-type male mice in the for forced swimming test, and showed enhanced inhibition in the Vogel's conflict model. These findings suggest that Dhh KO male mice exhibited enhanced anxiety and depressive behavior compared with wild-type male mice.
3. Role of Dhh in diabetic neuropathy
We found that Dhh mRNA levels are down regulated in sural nerves of human diabetic neuropathies.
4. Report of minifascicular neuropathy in Italy
We reported a case of polyneuropathy with minifascicular formation in Italy.

Report

(3 results)
  • 2006 Annual Research Report   Final Research Report Summary
  • 2005 Annual Research Report
  • Research Products

    (29 results)

All 2006 2005

All Journal Article (29 results)

  • [Journal Article] The structural and functional integrity of peripheral nerves depends on the glial-derived signal Desert Hedgehog.2006

    • Author(s)
      S.Sharghi-Namini, M.Turmaine, C.Meier, V.Sahni, Fujio Umehara, et al.
    • Journal Title

      J Neurosci 26(23)

      Pages: 6364-6376

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Elevated anxiety-like and depressive behavior in Desert hedgehog knockout male mice2006

    • Author(s)
      Fujio Umehara, Kenichi Mishima, Nobuaki Egashira, et al.
    • Journal Title

      Behavioural Brain Research 174(1)

      Pages: 167-173

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Annual Research Report 2006 Final Research Report Summary
  • [Journal Article] Relapsing cervical cord lesions on MRI in patients with HTLV-I-associated myelopathy.2006

    • Author(s)
      Fujio Umehara, Hiroyasu Tokunaga, Yoichi Hokezu, et al.
    • Journal Title

      Neurology 66(2)

      Pages: 289-289

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Annual Research Report 2006 Final Research Report Summary
  • [Journal Article] New mutation of gap junction protein beta1 (GJB1) gene in X-linked hereditary motor and sensory neuropathy.2006

    • Author(s)
      Fujio Umehara, Yumiko Arimura, Mitsuhiro Osame, et al.
    • Journal Title

      J Peripher Nerv Syst 11(1)

      Pages: 96-97

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] MRI studies of spinal visceral larva migrans syndrome.2006

    • Author(s)
      Fujio Umehara, Hideki Ookatsu, Daisuke Hayashi, et al.
    • Journal Title

      J Neurol Sci 249(1)

      Pages: 7-12

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Early clinical signs and imaging findings in Gerstmann-Straussler-Scheinker syndrome (Pro102Leu).2006

    • Author(s)
      Hitoshi Arata, Hiroshi Takashima, Fujio Umehara, et al.
    • Journal Title

      Neurology 66(11)

      Pages: 1672-1678

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Desert hedgehog-Patched 2 expression in peripheral nerves during Wallerian degeneration and regeneration.2006

    • Author(s)
      Sepideh N Bajestan, Fujio Umehara, Yuko Shirahama, et al.
    • Journal Title

      J Neurobiology 66(3)

      Pages: 243-255

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] The structural and functional integrity of peripheral nerves depends on the glial-derived signal Desert Hedgehog2006

    • Author(s)
      S.Sharghi-Namini, M.Turmaine, C.Meier, V.Sahni, Fujio Umehara, K.R.Jessen, R.Mirsky.
    • Journal Title

      J Neurosci 26

      Pages: 6364-6376

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Elevated anxiety-like and depressive behavior in Desert hedgehog knockout male mice.2006

    • Author(s)
      Fujio Umehara, Kenichi Mishima, Nobuaki Egashira, Ayumi Ogata, Katsunori Iwasaki, Michihiro Fujiwara.
    • Journal Title

      Behavioral Brain Research 174

      Pages: 167-173

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Relapsing cervical cord lesions on MRI in patients with HTLV-I-associated myelopathy.2006

    • Author(s)
      Fujio Umehara, Hiroyasu Tokunaga, Yoichi Hokezu, Ei Hokonohara, Koichi Yoshishige, Tadafumi Shiraishi, Ryuichi Okubo, Mitsuhiro Osame.
    • Journal Title

      Neurology 66

      Pages: 289-289

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] New mutation of gap junction protein beta1 (GJB1) gene in X-linked hereditary motor and sensory neuropathy.2006

    • Author(s)
      Fujio Umehara, Yumiko Arimura, Mitsuhiro Osame, S. Minato, Keiichi Nakahara.
    • Journal Title

      J Peripher Nerv Syst 11

      Pages: 96-97

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] MRI studies of spinal visceral larva migrans syndrome.2006

    • Author(s)
      Fujio Umehara, Hideki Ookatsu, Daisuke Hayashi, Akifumi Uchida, Yukari Douchi, H.Kawabata, Rina Goto, Akihiro Hashiguchi, Eiji Matsuura, Ryuichi Okubo, Itsuro Higuchi, Kimiyoshi Arimura, Y.Nawa, Mitsuhiro Osame.
    • Journal Title

      J Neurol Sci 249

      Pages: 7-12

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Early clinical signs and imaging findings in Gerstmann-Straussler-Scheinker syndrome (Pro102Leu).2006

    • Author(s)
      Hitoshi Arata, Hiroshi Takashima, Ryuki Hirano, Hisanori Tomimitsu, Koichi Machigashira, Kotaro Izumi, Mitsuro Kikuno, A.R. Ng, Fujio Umehara, Takayo Arisato, Ryuichi Ohkubo, Y. Nakabeppu, M. Nakajo, Mitsuhiro Osame, Kimiyoshi Arimura.
    • Journal Title

      Neurology 66

      Pages: 1672-1678

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Desert hedgehog-Patched 2 expression in peripheral nerves during Wallerian degeneration and regeneration.2006

    • Author(s)
      Sepideh N Bajestan, Fujio Umehara, Yuko Shirahama, et al.
    • Journal Title

      J Neurobiology 66

      Pages: 243-255

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Unilateral toe-walking secondary to intramuscular hemangioma in the gastrocnemius.2006

    • Author(s)
      Fujio Umehara, E.Matsuura, S.Kitajima, M.Osame.
    • Journal Title

      Neurology 65

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Motor-sensory neuropathy with minifascicle formation in a woman with normal karyotype2006

    • Author(s)
      A.landrini, S.Gambelli, M.Muglia, G.Berti, A.Patitucci, K.Sugie, F.Umehara, et al.
    • Journal Title

      Neurology 65

      Pages: 776-776

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Age associated axonal features in HNPP with 17p11.2 deletion in Japan2006

    • Author(s)
      H.Koike, M.Hirayama, M.Yamamoto, H.Ito, N.Hattori, Fujio Umehara, et al.
    • Journal Title

      J Neurol Neurosurg Psychiat 76

      Pages: 1109-1114

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] The structural and functional integrity of peripheral nerves depends on the glial-derived signal Desert Hedgehog.2006

    • Author(s)
      S.Sharghi-Namini, M.Turmaine, C.Mejer, V.Sahni, Fujio Umehara, K.R.Jessen, R.Mirsky.
    • Journal Title

      J Neurosci 26(23)

      Pages: 6364-6376

    • Related Report
      2006 Annual Research Report
  • [Journal Article] New mutation of gap junction protein beta1(GJB1) gene in X-linked hereditary motor and sensory neuropathy.2006

    • Author(s)
      Fujio Umehara, Yumiko Arimura, Mitsuhiro Osame, S. Minato, Keiichi Nakahara.
    • Journal Title

      J Peripher Nerv Syst 11(1)

      Pages: 96-97

    • Related Report
      2006 Annual Research Report
  • [Journal Article] MRI studies of spinal visceral larva migrans syndrome.2006

    • Author(s)
      Fujio Umehara, Hideki Ookatsu, Daisuke Hayashi, Akifumi Uchida, Yukari Douchi, H. et al.
    • Journal Title

      J Neurol Sci 249(1)

      Pages: 7-12

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Early clinical signs and imaging findings in Gerstmann-Straussler-Scheinker syndrome (Pro102Leu).2006

    • Author(s)
      Hitoshi Arata, Hiroshi Takashima, Ryuki Hirano, Fujio Umehara, et al.
    • Journal Title

      Neurology 66(11)

      Pages: 1672-1678

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Desert hedgehog-Patched 2 expression in peripheral nerves during Wallerian degeneration and regeneration.2006

    • Author(s)
      Sepideh N Bajestan, Fuiio Umehara, Yuko Shirahama, et al.
    • Journal Title

      J Neurobiology 66・3

      Pages: 243-255

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Relapsing cervical cord lesions on MRI in patients with HTLV-I-associated myelopathy.2006

    • Author(s)
      Fuiio Umehara, N Tokunaga, Y Hokezu, E Hokonohara, et al.
    • Journal Title

      Neurology 66・2

      Pages: 289-289

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Unilateral toe-walking secondary to intramuscular hemangioma in the gastrocnemius.2005

    • Author(s)
      Fujio Umehara, E.Matsuura, S.Kitajima, M.Osame
    • Journal Title

      Neurology 65(7)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Motor-sensory neuropathy with minifascicle formation in a woman with normal karyotype.2005

    • Author(s)
      A.Malandrini, S.Gambelli, M.Muglia, G.Berti, A.Patitucci, K.Sugie, F.Umehara, et al.
    • Journal Title

      Neurology 65(5)

      Pages: 776-776

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Age associated axonal features in HNPP with 17p 11.2 deletion in Japan.2005

    • Author(s)
      H.Koike, M.Hirayama, M.Yamamoto, H.Ito, N.Hattori, Fujio Umehara, et al.
    • Journal Title

      J Neurol Neurosurg Psychiat 76(8)

      Pages: 1109-1114

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Unilateral toe-walking secondary to intramuscular hemangioma in the gastrocnemius2005

    • Author(s)
      Fujio Umehara, E.Matsuura, S.Kitajima, M.Osame.
    • Journal Title

      Neurology 65・7

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Motor-sensory neuropathy with minifascicle formation in a woman with normal karyotype2005

    • Author(s)
      A.Malandrini, S.Gambelli, M.Muglia, G.Berti, A.Patitucci, K.Sugie, F.Umehara, et al.
    • Journal Title

      Neurology 65・5

      Pages: 776-776

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Age associated axonal features in HNPP with 17p11.2 deletion in Japan2005

    • Author(s)
      H.Koike, M.Hirayama, M.Yamamoto, H.Ito, N.Hattori, Fuiio Umehara, et al.
    • Journal Title

      J Neurol Neurosurg Psychiat 76・8

      Pages: 1109-1114

    • Related Report
      2005 Annual Research Report

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Published: 2005-04-01   Modified: 2016-04-21  

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