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Defect of carbohydrate-deficient glycoprotein syndrome type I

Research Project

Project/Area Number 09680604
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Structural biochemistry
Research InstitutionSASAKI INSTITUTE

Principal Investigator

OHKURA Takashi  SASAKI INSTITUTE, DEPT.OF BIOCHEMISTRY, 生化学部, 主任研究員 (50183223)

Project Period (FY) 1997 – 1998
Project Status Completed (Fiscal Year 1998)
Budget Amount *help
¥2,900,000 (Direct Cost: ¥2,900,000)
Fiscal Year 1998: ¥1,200,000 (Direct Cost: ¥1,200,000)
Fiscal Year 1997: ¥1,700,000 (Direct Cost: ¥1,700,000)
KeywordsCDGS / lipid-linked oligosaccharide / mannose / GPI-anchor / 遺伝病 / 糖蛋白質糖鎖不全 / フォスフォマンノムターゼ / デヒドロドリコールリダクターゼ / ドリコール
Research Abstract

The most characteristic abnormality of fibroblasts derived from CDGS-I patients is the truncated lipid-linked oligosaccharides (LLO), that are easily affected by glycoproteins and free mannose in FCS.Since the size of LLO from CDGS-I cells is normalized by a addition of mannose, mannose concentrations required for the normalization of LLO were analyzed.As a result, the LLOs of healthy control cells were normalized at 50% in about 7 muM of mannose, however, 28, 48, and 42 muM for CDGS-I cells.It indicates that CDGS-I in Japan is caused by a defect of mannose pathway, probably phosphomannomutase.

Report

(3 results)
  • 1998 Annual Research Report   Final Research Report Summary
  • 1997 Annual Research Report
  • Research Products

    (25 results)

All Other

All Publications (25 results)

  • [Publications] Yamashita,K.et al.: "Vip36の糖鎖認識と細胞内輸送における役割" 蛋白質核酸酵素. 43. 2455-2463 (1998)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Hara-Kuge,et al.: "Vesicular-integral membrane protein, VIP3,recognizes high-mannose type glycans containing α1→2 mannosyl residues in MDCK cells" Glycobiology in press.

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Yamashita,K.et al.: "Intracellular lectins associated with N-linked glycoprotein traffic" Biochem.Biochim.Acta in press.

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Fukushima,K.et al.: "Lectin-like characteristics of recombinant human interleukin-1β recognizing glycans of the glycosylphosphatidylinositol anchor" J.Biol.Chem. 272. 10579-10584 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Fukushima,K.et al.: "Synthesis of lipid-linked oligosaccharides is dependent on the cell cycle in rat 3Y1 cells" J.Biochem.(Tokyo). 121. 415-418 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Ohkura,T.et al.: "A partial deficiency of dehydrolichol reduction is a cause of carbohydrateglycoprotein syndrome type I" J.Biol.Chem. 272. 6868-6875 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Hara-Kuge, S., Ohkura, T., Seko, A., and Yamashita, K.: "VIP36 recognizes high-mannose type glycans in relation to apical membrane traffic." J.Cell.Biol.(submitted.).

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Fukushima, K., Hara-Kuge, S., Ohkura, T., Seko, A.Ideo, H., Inazu, T., and Yamashita, K.: "Lectin-like characteristics of recombinant human interleukin- Ibeta recognizing glycans of the glycosylphosphatidylinositol anchor." J.Biol.Chem.272. 10579-10584 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Fukushima, K., Ohkura, T., and Yamashita, K.: "Synthesis of lipid-linked oligosaccharides is dependent on the cell cycle in rat 3Y1 cells" J.Biochem. (Tokyo). 121. 415-418 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Ohkura, T., Fukushima, K., Kurisaki, A., Sagami, H., Ogura, K., Ohno, K., Hara-Kuge, S., and Yamashita, K.: "A partial deficiency of dehydrodolichol reduction is a cause of carbohydrate-glycoprotein syndrome type I." J.Biol.Chem.272. 6868-6875 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Hara-Kuge, S.Onkura, T.Seko A.and Yamashita K.: "Vesicularintegral membrane protein, VIP36, recognizes high-mannose type glycans containing alpha1*2 mannosyl residues in MDCK cells" Glycobiology. (in press.).

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Yoshikawa, K., Umetsu, K., Shinzawa, H., Yuasa, I., Ohkura, T., and Yamashita, K.: "Determination of carbohydrate-deficient transferrin separated by lectin affinity chromatography for detedting high alcohol consumption." Clin. Chem. (in press.).

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Hara-Kuge, S.Ohkura, T.and Yamashita K.: "VIP36 lectin activity associated with intracellular N-linked glycoprotein traffic." Glycoconjugate Journal. (in press.).

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Hara-Kuge, S.Ohkura, T.and Yamashita K.: "Co-distribution of VIP36 and N-linked glycoproteins associated with intracellular traffic in polarized MDCK cells." (Submitted for publication.).

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Yamashita,K.et al.: "VIP36の糖鎖認識と細胞内輸送における役割" 蛋白質核酸酵素. 43. 2455-2463 (1998)

    • Related Report
      1998 Annual Research Report
  • [Publications] Hara-Kuge, et al.: "Vesicular-integral membrane protein, VIP36, recognizes high-mannose type glycans containing α1→2 mannosyl residues in MDCK cells" Glycobiology. in press.

    • Related Report
      1998 Annual Research Report
  • [Publications] Tamashita,K.et al.: "Intracellular lectins associated with N-linked glycoprotein traffic" Biochem.Biochim.Acta. in press.

    • Related Report
      1998 Annual Research Report
  • [Publications] 福島慶子: "Synthesis of lipid-linked oligosaccharides is dependent on the cell cycle in rat 3Y1 cells." J.Biochem.(Tokyo). 121. 415-418 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] 大倉隆司: "A partial deficiency of dehydrodolichol reduction is a cause of carbohydrate-deficient glycoprotein syndrome typeI." J.Biol.Chem.272. 6868-6875 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] 福島慶子: "Lectin-like characteristics of recombinant human interleukin-1β recognizing glycans of glycosylphosphatidylinositol anchor." J.Biol.Chem.272. 10579-10584 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] 瀬古玲: "Quantitative differences in GlcNAc:β1→3 and GlcNAc:β1→4 galactosyltransferase activities between human colonic adenocarcinomas and normal colonic mucosa." Cancer Reserch. 56. 3468-3473 (1996)

    • Related Report
      1997 Annual Research Report
  • [Publications] 福島慶子: "Carbohydrate structures of a normal counterpart of the carcinoembryonic antigen produced by colon epithelial cells of normal adults" Glycobiology. 5. 105-115 (1995)

    • Related Report
      1997 Annual Research Report
  • [Publications] 山下克子: "Expression of Siaα2→6Galβ1→4GlcNAc residues on sugar chains of glycoproteins including carcinoembryonic antigens in human colon adenocarcinoma" Cancer Research. 55. 1675-1679 (1995)

    • Related Report
      1997 Annual Research Report
  • [Publications] 山下克子: "先天性N-結合型糖鎖転移不全症候群" 臨床検査. 39. 33-38 (1995)

    • Related Report
      1997 Annual Research Report
  • [Publications] 大倉隆司: "レクチンカラムクロマトグラフィー" 「グライコバイオロジー実験プロトコール」. 18-22 (1996)

    • Related Report
      1997 Annual Research Report

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Published: 1997-04-01   Modified: 2016-04-21  

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