• Search Research Projects
  • Search Researchers
  • How to Use
  1. Back to previous page

Analysis of genes for UDP-GT in Gilbert's sundrome and of neonatal cerebellar disorganization in model animals

Research Project

Project/Area Number 11670811
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionInstitute for Developmental Research, Aichi Human Service Center

Principal Investigator

KEINO Hiroomi  Institute for Development Research, Aichi Human Service Center, Dept. Pediatr., Section Chief, 周生期学部, 室長 (30090426)

Co-Investigator(Kenkyū-buntansha) KAWASIMA Sachiko  Institute for Development Research, Aichi Human Service Center, Dept. Pediatr., Tequnical Assistant, 周生期学部, 助手
Project Period (FY) 1999 – 2001
Project Status Completed (Fiscal Year 2001)
Budget Amount *help
¥3,600,000 (Direct Cost: ¥3,600,000)
Fiscal Year 2001: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 2000: ¥700,000 (Direct Cost: ¥700,000)
Fiscal Year 1999: ¥2,400,000 (Direct Cost: ¥2,400,000)
KeywordsBilirubin / amphoterin / BRUGT / Gilbert's / Crigler-Najjar / Gunn rat / cerebellum / granule neuron / Crigler-Najjar / Bilirubin / Gilberts / Glucuronosyltransferase / Neuroglycan C / NGC / Cerebellum / Purkinje cell / Rat
Research Abstract

Gilbert's syndrome is characterised by unconjugated hyperbilirubinemia due to partial absence of bilirubin UDP-glucuronosyltransferase. Nucleotide sequences of the genes for bilirubin UDPglucuronosyltransferase were analysed in umbilical blood. Some babies had a missense mutations coused by a single nucliotide substitution and the mutations were heterozygous. The babies had an abnormality in the promoter region of the gene (estra TAin TATAA box). In addition, relatives of patients with Crigler-Najjar syndrome Type I and II, and those with Gilbert's syndrome were analysed. All relatives with mild hyperbilirubinemia were heterozygotes with respect to each defective allele. These results suggest that Gilbert's syndrome is inherited as a dominant trait.
Proteoglycans are some of the major constituents of the extra cellular matrix and cell membranes. They are located at cell surface and extra cellular space, not only in connective tissues but also in central nervous system. In developing brain, neuroglycan C, neurocan, neuregulin, and amphoterin are presnt and preisely regulated. The structure of neuroglycan C changes from a proteoglycan form to a nonproteoglycan form without chondroiti sulfate side chanis as the cerebellar development proceeds. The rapid appearance of chondroitinsulfate conjugated neuregulin was observed in the adult rat brain which was surgically injured. The poly saccharide side chain may play important roles in various cellular processes such as cell proliferation and neuronal circuits formation. It is also showed that the subsets of suckling rat cerebellar neurons possess different classes of oligosaccharide conjugated amphoterin, and their conjugation dramatically changes during the developmental prograss. In the nucleus of external granule neuron, oligosaccharide conjugated amphoterin strongly combined with lectin-like proteins, then the complex may combined with DNA. The oligosaccaride-free amphoterin are soluble in the matrix of nucleus.

Report

(4 results)
  • 2001 Annual Research Report   Final Research Report Summary
  • 2000 Annual Research Report
  • 1999 Annual Research Report
  • Research Products

    (21 results)

All Other

All Publications (21 results)

  • [Publications] S.Aono et al.: "Genomic organization and expression pattern of mouse neuroglycan C in the cerebellar development"J Biol Chem. 275. 337-342 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] M.Matsuda, H.Keino: "Roles of β-catenin in inner ear development in rat embryos"Anat Embryol. 202. 39-48 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] T.nakatsuji et al.: "Changes in the mounts of the molt-inhibiting hormone glands during the molt cycle of the American cryfish"Zool Sci. 17. 1129-1136 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] M.Matsuda, H.Keino: "Possible roles of β-catenin in evagination of the optic primordiium in rat embryos"Develop Growth Differ. 43. 391-400 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Y.Tokita et al.: "Regulation of neuregulin expression in the injured rat brain and cultured astrocytes"J Neurosci. 21. 1257-1264 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] 慶野宏臣, 青野幸子: "日本遺伝子治療学会編 遺伝子治療開発研究ハンドブック"エヌ・ティー・エス社. 1061 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] M. Matsuda & H. Keino: "Roles of β-catenin in inner ear development in rat embryos"Anat Embryol. 202. 39-48 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] S. Aono, H. Keino, T. Ono, Y. Yasuda, Y. Tokita, F. Matsui, M. Taniguchi, S. Sonta, A. Oohira: "Genomic organization and expression pattern of mouse neuroglycan C in the cerebellar development"J Biol Chem. 275. 337-342 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] T. Nakatsuji, H. Keino, T. Tamura, S. Yoshimura, T. Kawakami, S. Aimoto, H. Sonobe: "Changes in the mounts of the molt-inhibiting hormone in sinus glands during the molt cycle of the American cryfish, Procambarus clarkii"Zool Sci. 17. 1129-1136 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] M. Matsuda & H. Keino: "Possible roles of β-catenin in evagination of the optic primordiium in rat embryos"Develop Growth Differ. 43. 391-400 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Y. Tokita, H. Keino, F. Matsui, S. Aono, H. Ishiguro, S. Higashiyama, A. Oohira: "Regulation of neuregulin expression in the injured rat brain and cultured astrocytes"J Neurosci. 21. 1257-1264 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] S.Aono et al.: "Genomic organization and expression pattern of mouse neuroglycan C in the cerebellar development"J Biol Chem. 275. 337-342 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] M.Matsuda, H.Keino: "Roles of β-catenin in inner ear development in rat embryos"Anat Embryol. 202. 39-48 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] T.Nakatsuji et al.: "Changes in the mounts of the molt-inhibiting hormone in sinus glands during the molt in rat embryos"Zool Sci. 17. 1129-1136 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] M.Matsuda, H.Keino: "Possible roles of β-catenin in evagination of the optic I primordiium in rat embryos"Develop Growth Differ. 43. 391-400 (2000)

    • Related Report
      2001 Annual Research Report
  • [Publications] Y.Tokita et al.: "Regulation of neuregulin expression in the injured rat brain and cultured astrocytes"J Neurosci. 21. 1257-1264 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] 慶野宏臣, 青野幸子: "日本遺伝子治療学会編 遺伝子治療開発研究ハンドブック"エヌ・ティー・エス社. 1061 (1999)

    • Related Report
      2001 Annual Research Report
  • [Publications] M.Matsuda & H.Keino: "Roles of β-catentin in inner ear development in rat embryos"Anatomy and Embryology. 202. 39-48 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] T.Nakatsuji, et al: "Changes in the amounts of the molt-inhibiting hormone in sinus glands during the molt cycle of the american crayfish, Procambarus clarkii"Zoological Science. 17. 1129-1136 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] S. Aono, H. Keino et al.: "Genomic organization and expression pattern of mouse neuroglycan C in the cerebellar development"The Journal of Biological Chemistry. 275, 1. 337-342 (2000)

    • Related Report
      1999 Annual Research Report
  • [Publications] 慶野宏臣 他: "遺伝子治療開発研究ハンドブック"日本遺伝子治療学会. 1061 (1999)

    • Related Report
      1999 Annual Research Report

URL: 

Published: 1999-04-01   Modified: 2016-04-21  

Information User Guide FAQ News Terms of Use Attribution of KAKENHI

Powered by NII kakenhi