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Research on the pathophysiology of human dysmyelination using magnetic resonance imaging of a mutant mouse

Research Project

Project/Area Number 15591084
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionIbaraki Prefectural University of Healty Sciences (2004)
University of Tsukuba (2003)

Principal Investigator

IWASAKI Nobuaki  Ibaraki Prefectural University of Healty Sciences, 付属病院, Associate professor (70251006)

Co-Investigator(Kenkyū-buntansha) MATSUMURA Akira  Institute of Clinical Medicine, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Department of Neurosurgery, Professor (90241819)
HOMMA Kazuhiro  Ibaraki Prefectural University of Healty Sciences, The National Institute of Advanced Industrial Science and Technology, Institute for Human Science & Biomedical Engineering, Group reader (80357970)
Project Period (FY) 2003 – 2004
Project Status Completed (Fiscal Year 2004)
Budget Amount *help
¥3,000,000 (Direct Cost: ¥3,000,000)
Fiscal Year 2004: ¥1,000,000 (Direct Cost: ¥1,000,000)
Fiscal Year 2003: ¥2,000,000 (Direct Cost: ¥2,000,000)
Keywordsmyelination / dysmyelination / myelin basic protein / magnetic resonance imaging / mutant mouse / shiverer mouse / 18q-syndrome / 磁気共鳴画像法 / ミュータントマウス
Research Abstract

A magnetic resonance imaging technique was developed in which the saddle coil was adjusted for use on the mouse brain. This technique was applied to the shiverer mutant mouse, which has an altered myelin basic protein (MBP) gene. The T1 and T2 weighted images were acquired and T1 and T2 maps were calculated. In the wild-type mice and the heterozygous mutant mice (MBPshi/+), the corpus callosum and the radiation of the corpus callosum displayed low intensity signal on T2 weighted images. The basal ganglia also displayed slightly lower intensity signal. On the other hand, the shiverer mutant mice (MBPshi/MBPshi) did not show a clear difference between the signal intensities of the cerebral cortex and the white matter.
Kluver-Barrera (KB) staining, immunohistochemistry with anti-MBP antibody and electron micrographs were performed on the corpus callosum of wild-type, MBPshi/+ and MBPshi/MBPshi mice. No difference in the myelin staining, nor in the multilamellar membrane structures of myeli … More n sheaths was revealed between the wild-type and the MBPshi/+ mice. Therefore, the MBPshi/+ mice with only one copy of the abnormal MBP gene were not found to be a valid model of human 18q- syndrome.
A boy afflicted with chromosome 18 ring was investigated by brain MRI and autopsy examination. The T2 weighted image revealed diffuse high intensity signals throughout the cerebral white matter. Immunohistochemical examination showed uniform KB and anti-MBP staining of the cerebral white matter. Electron microscopy revealed clusters of axons wrapped by compacted myelin sheaths with distinct major dense lines. It has previously been assumed that the high intensity signal of T2 weighted MRI images was caused by dysmyelination resulting from the MBP gene abnormality. Our pathological study demonstrated that the cerebral white matter was well-myelinated, a result which is quite different from the current understanding of 18q-syndrome.
We show the importance of using pathophysiological analysis in combination with MRI imaging in evaluating this mutant mouse as a valid animal model. Less

Report

(3 results)
  • 2004 Annual Research Report   Final Research Report Summary
  • 2003 Annual Research Report
  • Research Products

    (8 results)

All 2008 2006 2005

All Journal Article (8 results)

  • [Journal Article] 水励起高速三次元拡散強調MRIパルスシーケンスの開発とラット骨盤部への生体応用2008

    • Author(s)
      沼野智一, 本間一弘, 岩崎信明, 兵頭行志, 新田尚隆
    • Journal Title

      日本磁気共鳴医学会雑誌 28・3

      Pages: 185-195

    • NAID

      10025696274

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Diffusion-weighted imaging of the rat pelvis using 3D water-excitation MP-RAGE sequence(in Japanese)2008

    • Author(s)
      Numano T, Homma K, Iwasaki N, Nitta N
    • Journal Title

      Japanese Journal of Magnetic Resonance in Medicine 28-3

      Pages: 185-195

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Magnetic resonance imaging of the brain and haematological profile in adult cyanotic congenital heart disease without stroke2006

    • Author(s)
      Horigome H, Iwasaki N, Anno I, Kurachi S, Kurachi K
    • Journal Title

      Heart 92・2

      Pages: 263-5

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] In vivo isotropic 3D diffusion tensor mapping of the rat brain using diffusion-weighted 3D MP-RAGE MRI2006

    • Author(s)
      Numano T, Homma K, Iwasaki N, Hyodo K, Nitta N, Hirose T
    • Journal Title

      Magn Reson Imaging 24・3

      Pages: 287-93

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Magnetic resonance imaging of the brain and haematological profile in adult cyanotic congenital heart disease without stroke2006

    • Author(s)
      Horigome H, Iwasaki N, Anno I, Kurachi S, Kurachi K
    • Journal Title

      Heart 92-2

      Pages: 263-5

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] In vivo isotropic 3D diffusion tensor mapping of the rat brain using diffusion-weighted 3D MP-RAGE MRI2006

    • Author(s)
      Numano T, Homma K, Iwasaki N, Hyodo K, Nitta N, Hirose T
    • Journal Title

      Magn Reson Imaging 24-3

      Pages: 287-93

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] 脂肪抑制高速三次元拡散強調MRIの開発と生体応用2005

    • Author(s)
      沼野智一, 本間一弘, 高橋信幸, 廣瀬武志
    • Journal Title

      日本磁気共鳴医学会雑誌 25・2

      Pages: 108-117

    • NAID

      10016157771

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Fat-saturated diffusion-weighted imaging with three-dimensional MP-RAGE sequence(in Japanese)2005

    • Author(s)
      Numano T, Homma K, Takahashi N, Hirose T
    • Journal Title

      Japanese Journal of Magnetic Resonance in Medicine 25-2

      Pages: 108-117

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary

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Published: 2003-04-01   Modified: 2016-04-21  

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