Investigation for the pathogenesis of neurological disorders due to Desert hedgehog gene mutation and development of new treatment

2008 Fiscal Year Final Research Report

• Project/Area Number: 19591000
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Single-year Grants
• Section: 一般
• Research Field: Neurology
• Research Institution: Kagoshima University
• Principal Investigator: UMEHARA Fujio Kagoshima University, 大学院医歯学総合研究科, 講師 (20271140)
• Co-Investigator(Kenkyū-buntansha): HASHIGUCHI Teruto 鹿児島大学, 大学院・医歯学総合研究科, 准教授 (70250917) ; ARIMURA Kimiyoshi 鹿児島大学, 大学院・医歯学総合研究科, 准教授 (30140908)
• Project Period (FY): 2007 – 2008
• Keywords: Desert hedgehog / 末梢神経障害 / 遺伝子治療 / シュワン細胞 / 遺伝子変異

Research Abstract

ヒトDHH遺伝子発現プラズミドの作成に成功し、COS細胞への遺伝子導入によるDHH蛋白の産生・分泌を確認したこと。さらに、このプラズミドをラット坐骨神経損傷モデルにおいて、intraneural injection を行い、その効果を検討した。ラット末梢神経損傷モデルにおいて、pHDHHの神経束内注入法では、一部には再生促進効果がみられたものの、対象に比べ統計学的に優位な再生促進効果は確認できていない。より有効な投与方法の改良が今後の課題である。

Research Products

• [Journal Article] Enlarged multifocal upper limb neuropathy with HTLV-I associated myelopathy in a patient with chronic adult T-cell leukemia (2008)
  • Author(s): Umehara, F. Hagiwara, T. Yoshimura, M. Higashi, K. Arimura, K.
  • Journal Title: J Neurol Sci 266 Pages: 167-170
  • Peer Reviewed
• [Journal Article] Accumulation of human T-lymphotropic virus type I (HTLV-I)-infected cells in the cerebrospinal fluid during the exacerbation of HTLV-I-associated myelopathy (2008)
  • Author(s): Hayashi D, Kubota R, Takenouchi N, Nakamura T, Umehara F, Arimura K, Izumo S, Osame M.
  • Journal Title: J Neurovirol 14 Pages: 459-63
  • Peer Reviewed
• [Journal Article] Expression of the heparin-binding growth factor midkine in the cerebrospinal fluid of patients with neurological disorders (2008)
  • Author(s): Yoshida Y, Ikematsu S, Muramatsu H, Sakakima H, Mizuma N, Matsuda F, Sonoda K, Umehara F, Ohkubo R, Matsuura E, Goto M, Osame M, Muramatsu T.
  • Journal Title: Intern Med 47 Pages: 83-89
  • Peer Reviewed
• [Journal Article] Inclusion body myositis associated with human T-lymphotropic virus-type I infection: eleven patients from an endemic area in Japan (2008)
  • Author(s): Matsuura E, Umehara F*, Nose H, Higuchi I, Matsuoka E, Izumi K, Kubota R, Saito M, Izumo S, Arimura K, Osame M.
  • Journal Title: J Neuropathol Exp Neurol 67 Pages: 41-49
  • Peer Reviewed
• [Journal Article] POEMS 症候群(Crow-Fukase 症候群) (2008)
  • Author(s): 梅原藤雄、有村公良
  • Journal Title: Clinical Neuroscience 26 Pages: 244-245
• [Journal Article] 自己免疫性末梢神経障害血液浄化療法-2008 (2008)
  • Author(s): 梅原藤雄
  • Journal Title: 腎と透析 第65巻増刊号 Pages: 454-458
• [Journal Article] Spinocerebellar ataxia with axonal neuropathy : consequence of a Tdp1 recessive neomorphic mutation (2007)
  • Author(s): Hirano R, Interthal H, Huang C, Nakamura T, Deguchi K, Choi K, Bhattacharjee MB, Arimura K, Umehara F, Izumo S, Northrop JL, Salih MA, Inoue K, Armstrong DL, Champoux JJ, Takashima H, Boerkoel CF
  • Journal Title: EMBO J. 26 Pages: 4732-4743
  • Peer Reviewed
• [Journal Article] Abnormalities of spinal magnetic resonance images implicate clinical variability in human T-cell lymphotropic virus type I-associated myelopathy (2007)
  • Author(s): Umehara F, Nose H, Saito M, Fukuda M, Ogino M, Toyota T, Yuhi T, Arimura K, Osame M.
  • Journal Title: J Neurovirol 13 Pages: 260-267
  • Peer Reviewed
• [Journal Article] 末梢神経障害の治療の進歩 (2007)
  • Author(s): 梅原藤雄、有村公良
  • Journal Title: 神経治療学 24 Pages: 445-448