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EXPERIMENTAL STUDY FOR NEURONAL MIGRATION DISORDERS DUE TO BARRIER DEFECTS OF THE PIAL-GLIAL BORDER: INTENTION OF ANIMAL MODEL CONFIRMATION OF THE BRAIN MALFORMATIONS IN FUKUYAMA CONGENITAL MUSCULAR DYSTROPHY

Research Project

Project/Area Number 63570437
Research Category

Grant-in-Aid for General Scientific Research (C)

Allocation TypeSingle-year Grants
Research Field Pediatrics
Research InstitutionTOTTORI UNIVERSITY

Principal Investigator

TAKADA Kuniyasu  DEPARTMENT OF MEDICINE, ASSOCIATE PROFESSOR, 医学部, 助教授 (00105909)

Co-Investigator(Kenkyū-buntansha) KAWAHARA Hitoshi  DEPARTMENT OF MEDICINE, ASSISTANT, 医学部・附属病院, 助手 (00186123)
HOUDOU Sadataka  NATIONAL INSTITUTE OF NEUROSCIENCE, NCNP, RESEARCH FELLOW, 神経センター神経研究所疾病研究第二部, 非常勤流動研究員 (70192317)
Project Period (FY) 1988 – 1989
Project Status Completed (Fiscal Year 1989)
Budget Amount *help
¥1,400,000 (Direct Cost: ¥1,400,000)
Fiscal Year 1989: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 1988: ¥900,000 (Direct Cost: ¥900,000)
KeywordsFUKUYAMA CONGENITAL MUSCULAR DYSTROPHY / CEREBRAL CORTEX, ABNORMALITIES / FETAL PERIOD / MIGRATION OF NEURONAL CELLS / PIAL-GLIAL BARRIER / PIA MATER / 大脳皮質,形成異常 / グリア-神経細胞の発生 / 軟膜間葉系と神経上皮の相互作用 / 福山型先天性筋ジストロフィー / 大脳皮質形成異常 / グリア限界膜 / 脳軟膜
Research Abstract

In order to make clear the pathogenesis of the unique cerebral malformation in Fukuyama type congenital muscular dystrophy (FCMD) according to our own hypothesis based on neuropathological observations, experiments are planned to induce in newborn or late fetal rodents glio-mesenchymal proliferation in the subarachnnoidal spaces and focal defects of pial-glial (or leptomeningo- glioneural) barriers, and subsequently similar migration disorders in FCMD. Human autopsy materials of FCMD including a fetal case are also reevaluated neuropathologically.
At first we intended focal cold injuries in the newborn rat cerebrum, because migration disorders are often concomitant in the cortex around the human porencephalic cysts. Although focal cortical defects similar to human porencephaly were induced, gyral or cytoarchitectural abnormalities were unsuccessful. In the next step, we try to induce cerebellar dysplasia (micropoly- gyria) by intracisternal administration to newborn rats of a neurotoxic … More agent, 6-hydroxydopamine, by which some investigators have succeeded to induce abnormal cerebellar cortex. In our experiment, focal meningeal thicking and hydrocephalus were induced in a few cases, but true cerebellar cortical dysplaga has not been made. The experiment is still continued.
Human autopsy cases were reevaluated by a Golgi and angioarchitectural study. the results suggested that in the loci with minor dysplasia, abnormal neural accumulation is mainly formed around long penetrating vessels, while in the severest malformation the cerebral cortex also consisted of two heterogeneous layers; a supportive finding of massive accumulation of migrating neurons in the thickened glio-mesenchymal layer.
Analysis of fetal cerebellar cortex suggested that, although mesenchymal proliferation in the fetal period is important as well as in the cerebral cortex, but interruption of cortical components including precursors of granular or Purkinje cells by proliferated mesencynal tissue is rather essential for development of cerebellar cortical dysplasia, instead of glial-mesenchymal intermixing seen in the cerebrum.
Our results obtained from animal experiments are still minimal, but human neuropathological analysis, including fetal case, is significant. We hope to continue to elucidate the pathogenesis of this peculiar brain malformation, which is particularly common in Japan. Less

Report

(3 results)
  • 1989 Annual Research Report   Final Research Report Summary
  • 1988 Annual Research Report
  • Research Products

    (14 results)

All Other

All Publications (14 results)

  • [Publications] Takada K,Becker LE,Chan F: "Aberrant dendritic development in the human agyric cortex:a quantitative and qualitative Golgi study of two cases" Clin Neuropathol. 7. 111-119 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Takada K: "Fukuyama congenital muscular dystrophy as a unique disorder of neuronal migration:a neuropathological review and hypothesis" Yonago Acta med. 31. 1-66 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Takada K,Shiota M,Ando M,Kimura M,Inoue K: "Porencephaly and hydranencephaly:a neuropathological study of four autopsy cases" Brain Dev(Tokyo). 11. 51-56 (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] 高田邦安: "小多脳回Micropolygyria" Clinical Neuroscience. 9. 950-951 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Takada K, Nakamura H, Takashima S: "Cortical dysplasia in Fukuyama congenital muscular dystrophy (FCMD): a Golgi and angioarchitectonic analysis" Acta Neuropathol (Berl). 76. 170-178 (1988)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Takada K, Becker LE, Chan F: "Aberrant dendritic development in the human agyric cortex: a quantitative and qualitative Golgi study of two cases" Clin Neuropathol 1988; 7: 111-119.

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Takada K: "Fukuyama congenital muscular dystrophy as a unique disorder of neuronal migration: a neuropathological review and hypothesis" Yonago Acta med 1988; 31: 1-16.

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Takada K, Shiota M, Ando M, Kimura M, Inoue K: "Porencephaly and hydranencephaly: a neuropathological study of four autopsy cases" Brain Dev (Tokyo) 1989; 11: 51-56.

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Takada K, Nakamura H: "Cerebellar micropolygyria in Fukuyama congenital muscular dystrophy: observation in the fetal and postnatal cases"

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Takada K, Nakamura H,Takashima S: "Cortical dysplasia in Fukuyama congenital muscular dystrophy(FCMD):a Golgi and angioarchitectonic analysis" Acta Nauropathol(Berl). 76. 170-178 (1988)

    • Related Report
      1989 Annual Research Report
  • [Publications] Takada K,Becker LE,Chan F: "Aberrant dendritic development in the human agyric cortex:a quantitative and qualitative Golgi study of two cases" Clin Neuropathol. 7. 111-119 (1988)

    • Related Report
      1989 Annual Research Report
  • [Publications] Takada K,Shiota M,Ando M,Kimura M,lnoue K: "Porencephaly and hydranencephaly:a neuropathological study of four autopsy cases" Brain Dev(Tokyo). 11. 51-56 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] Takada K,Nakamura H: "Cerebellar micropolygyria in Fukuyama congenital muscular dystrophy:observation in the fetal and postnatal cases"

    • Related Report
      1989 Annual Research Report
  • [Publications] Kuniyasu Takada.;Haruomi Nakamura.;Sachio Takashima.: Acta Neuropathol.(Berl). 76. 170-178 (1988)

    • Related Report
      1988 Annual Research Report

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Published: 1988-04-01   Modified: 2016-04-21  

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