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Role of Rab38G protein expressed in specifically in the lower respiratory epithelium in the interstitial lung disease

Research Project

Project/Area Number 15590833
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Respiratory organ internal medicine
Research InstitutionKanazawa Medical University

Principal Investigator

OSANAI Kazuhiro  Kanazawa Medical University, Dept Medicine, Associate Professor (70221158)

Co-Investigator(Kenkyū-buntansha) 谷口 豊  金沢医科大学, 医学部, 助教授 (70125863)
Project Period (FY) 2003 – 2006
Project Status Completed (Fiscal Year 2006)
Budget Amount *help
¥3,300,000 (Direct Cost: ¥3,300,000)
Fiscal Year 2006: ¥700,000 (Direct Cost: ¥700,000)
Fiscal Year 2005: ¥600,000 (Direct Cost: ¥600,000)
Fiscal Year 2004: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 2003: ¥1,200,000 (Direct Cost: ¥1,200,000)
KeywordsRab38 small GTPase / Interstitial Lung Disease / Lune surfactant / Hermansky-Pudlak syndrome / Rab38低分子量Gタンパク質 / ルビーラット / Rab38 / 低分子量Gタンパク質 / 肺胞II型上皮細胞 / 白皮症 / 出血異常 / サーファクタント / 肺気腫 / チョコレートマウス
Research Abstract

Ruby rats including Fawn-Hooded and Long Evans Cinnamon/Crj (LEC/Crj) are known as animal model of Hermansky-Pudlak syndrome. We extracted genome DNA from tails of these rats, performed DNA sequence, and found a point mutation in the translation initiation codon in the exon 1 of Rab38 gene, resulting in ATG to ATA that indicates no translation of nucleic acids to amino acids. Western blot analysis on the lung tissue lysates from these rats confirmed no existence of Rab38 protein. These rats had ruby-like dark red eyes and cinnamon-like coat hairs, and showed elongation of bleeding time to more than 15 minutes. Thus, these rats have oculocutaneous albinism. The lungs of LEC/Crj showed emphysema-like appearance, and alveolar type II cells were swelled with cytoplasmic lammelar bodies increased in number and in size. Western blot analysis of bronchoalveolar lavage fluid, lamellar body fraction, and total lung homogenate showed that surfactant protein B was increased in the lung tissue but decreased in the alveolar lumen. Isolated alveolar type II cells from LEC/Crj showed decreased basal secretion of [3H]-rabeled surfactant phosphatidylcholine but remarkably increased agonist-induced secretion. These results indicate that Rab38 deficiency in the rat causes oculocutaneous albinism, bleeding diathesis, and lung disease. As these phenotypes correspond to those of human Hermansky-Pudlak syndrome, it is suggested that genetic abnormality of Rab38 may cause the disease in humans.

Report

(5 results)
  • 2006 Annual Research Report   Final Research Report Summary
  • 2005 Annual Research Report
  • 2004 Annual Research Report
  • 2003 Annual Research Report
  • Research Products

    (39 results)

All 2008 2007 2006 2005 2004 2003

All Journal Article (39 results)

  • [Journal Article] Chocolateマウスに発現される変異Rab38タンパク質(Rab38^<cht>)の特性解析2008

    • Author(s)
      長内 和弘
    • Journal Title

      分子呼吸器病 12

      Pages: 61-64

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] chcolate ムウスに発現される変異Rab38 タンパク質(Rab38_cht)の特性解析2008

    • Author(s)
      長内和弘
    • Journal Title

      分子呼吸器病 12

      Pages: 61-64

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Melanocyote differentiation antigen RAB38/NY-MEL-1 induces frequent antibody responses exclusively in melanoma patients2007

    • Author(s)
      Zippelius A
    • Journal Title

      Cancer Immunol Immunother 56

      Pages: 249-258

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Rab38欠損ラットにおける肺病変の検討2007

    • Author(s)
      長内 和弘
    • Journal Title

      分子呼吸器病 11

      Pages: 61-63

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Melanocyte differentiatior antigen RAB38/NY-MEL-1induces frequent antibody responses exclusively in melanoma patients.2007

    • Author(s)
      Affled, Zippelius.
    • Journal Title

      Cancer Immunol Immunother. 56

      Pages: 249-58

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Rab38欠損ラットにおける肺病変の検討2007

    • Author(s)
      長内和弘
    • Journal Title

      分子呼吸器病 11

      Pages: 61-63

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Rab38欠損ラットにおける肺病変の検討2007

    • Author(s)
      長内和弘, 他
    • Journal Title

      分子呼吸器病 11・1

      Pages: 61-63

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Pulmonary surfactant transport in alveolar type II cells2006

    • Author(s)
      Kazuhiro Osanai
    • Journal Title

      Respirology 11

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Stimulation of alveolar epithelial fluid clearance in human lungs by exogenous epinephrine2006

    • Author(s)
      Tsutomu Sakukma
    • Journal Title

      Crit Care Med 34

      Pages: 676-681

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Rab38低分子量Gタンパク質の遺伝子突然変異マウスにおける肺サーファクタント輸送異常と気腫性肺病変2006

    • Author(s)
      長内 和弘
    • Journal Title

      分子呼吸器病 10

      Pages: 191-194

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] II型肺胞上皮細胞における細胞内蛋白移送と関連疾患の検討2006

    • Author(s)
      長内 和弘
    • Journal Title

      分子呼吸器病 11

      Pages: 155-159

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] 肺サーファクタントの細胞内処理過程の障害によって引き起こされる肺疾患2006

    • Author(s)
      長内 和弘
    • Journal Title

      日本界面医学会雑誌 37

      Pages: 4-6

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Pulmonary surfactant transport in alveolar type II cells.2006

    • Author(s)
      Kazuhiro, Osanai.
    • Journal Title

      Respirology 11

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Stimulation of alveolar epithelial fluid clearance in human lungs by exogenous epinephrine.2006

    • Author(s)
      Tsutomu, Sakukma.
    • Journal Title

      Crit Care Med. 34

      Pages: 676-681

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Rab38低分子量Gタンパク質の遺伝子突然変異ムウスにおける肺サーファクタント輸送変異ムウスにおける肺サーファクタント輸送異常と気腫性肺病変2006

    • Author(s)
      長内和弘
    • Journal Title

      分子呼吸器病 10

      Pages: 191-194

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] II型肺胞上皮細胞における細胞内蛋白移送と関連疾患の検討2006

    • Author(s)
      長内和弘
    • Journal Title

      分子呼吸器病 11

      Pages: 155-159

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] 肺サーフアクタントの細胞内処理過程の障害によって引き起こされる肺疾患2006

    • Author(s)
      長内和弘
    • Journal Title

      日本界面医学会雑誌 37

      Pages: 4-6

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] 肺サーファクタントの細胞内処理過程における障害によって引き起こされる肺疾患2006

    • Author(s)
      長内和弘
    • Journal Title

      日本界面医学会雑誌 37・1,2

      Pages: 4-6

    • NAID

      10018297910

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Rab38低分子量Gタンパク質の遺伝子突然変異マウスにおける肺サーファクタント輸送異常と気腫性肺病変2006

    • Author(s)
      長内和弘, 他
    • Journal Title

      分子呼吸器病 10・3

      Pages: 47-50

    • Related Report
      2006 Annual Research Report
  • [Journal Article] II型肺胞上皮細胞における細胞内蛋白移送と関連疾患の検討2006

    • Author(s)
      長内和弘
    • Journal Title

      分子呼吸器病 10・3

      Pages: 11-15

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Pulmonary surfactant transport in alveolar type II calls2006

    • Author(s)
      Kazuhiro Osanai, et al.
    • Journal Title

      Respirology 11

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Pulmonary surfactant transport in alveolar type II cells2006

    • Author(s)
      Kazuhiro Osanai, et al.
    • Journal Title

      Respirology 11・Jan

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Expression and characterization of Rab38,a new member of the Rab small G protein family2005

    • Author(s)
      Kazuhiro Osanai
    • Journal Title

      Biol Chem 386

      Pages: 143-153

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] マウスにおけるRab38点突然変異は眼皮膚型白皮症と気腫性肺病変を引き起こす2005

    • Author(s)
      八田 理恵子
    • Journal Title

      日本界面医学会雑誌 36

      Pages: 15-17

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] プロテアーゼ・アンチプロテアーゼ説2005

    • Author(s)
      井口 晶晴
    • Journal Title

      Geriateric Medicine(老年医学) 43

      Pages: 405-409

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Expression and characterizatioin of Rab38, a new member of the Rab small G protein family.2005

    • Author(s)
      Kazuhiro, Osanai.
    • Journal Title

      3iol. Chem. 386

      Pages: 143-153

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] マウスにおけるRab38点突然変異は眼皮膚型と気腫性変異を引き起こす2005

    • Author(s)
      八田理恵子
    • Journal Title

      日本界面医学会雑誌 36

      Pages: 15-17

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] プロテアーゼアンチプロテアーゼ説2005

    • Author(s)
      井口晶晴
    • Journal Title

      Geriatric Medicine(老年医学) 43

      Pages: 405-409

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Expression and characterization of Rab38, a new member of the Rab small G protein family2005

    • Author(s)
      Kazuhiro Osanai et al.
    • Journal Title

      Biological Chemistry 386・Feb

      Pages: 143-153

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Effects of α1-,β1-,β2-,β3-adrenergic/agonists on alveolar fluid clearance in isolated rat lungs2004

    • Author(s)
      Xio Gu
    • Journal Title

      Journal of Kanazawa Medical University 29

      Pages: 91-96

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Role of CXC chemokines in ventilator-induced lung injury in rats2004

    • Author(s)
      Hirohisa Toga
    • Journal Title

      J Jpn Med Soc Biol Interface 35

      Pages: 9-16

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] 肺胞II型上皮細胞における肺サーファクタントの輸送経路2004

    • Author(s)
      長内 和弘
    • Journal Title

      分子呼吸器病 8

      Pages: 235-241

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Effects of al-, bi-, b2-, b3-AdrenergicAgonists on Alveolar Fluid Clearance in Isolated Rat Lungs.2004

    • Author(s)
      Xio, Gu.
    • Journal Title

      Kanazawa Med. Univ. 29

      Pages: 91-96

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Role of CXC chemokines in ventilator-induced lung injury in rats.2004

    • Author(s)
      Hirohisa, Toga.
    • Journal Title

      J. Jpn. Med. Soc. Biol. Interface. 35

      Pages: 9-16

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] 肺胞II型上皮細胞における肺サーファクタントの輸送経路2004

    • Author(s)
      長内和弘
    • Journal Title

      分子呼吸器病 8

      Pages: 235-241

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Mechanical stress on type II cells and production of CXC chemokine2003

    • Author(s)
      Hirohisa Toga
    • Journal Title

      J Jpn Med Soc Biol Interface 34

      Pages: 77-80

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] 気道炎症にかかわる酸化ストレスの関与2003

    • Author(s)
      長内 和弘
    • Journal Title

      日本臨床 61

      Pages: 2119-2124

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Mechanical stress on type II cells and production of CXC chemokine.2003

    • Author(s)
      Hirohisa, Toga.
    • Journal Title

      J. Jpn. Med. Soc. Biol. Interface. 34

      Pages: 77-80

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] 気道炎症にかかわる酸化ストレスの関与2003

    • Author(s)
      長内和弘
    • Journal Title

      日本臨床 61

      Pages: 2119-2124

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary

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

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