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A study on pathogenesis of idiopathic interstitial pneumonia : Using ep mice, a mouse model of Hermansky-pudlak syndrome.

Research Project

Project/Area Number 14570197
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Experimental pathology
Research InstitutionYokohama City University

Principal Investigator

NAKATANI Yukio  Yokohama City University Hospital, Associate Professor, 医学部附属病院, 助教授 (20137037)

Co-Investigator(Kenkyū-buntansha) TANG Xiaoyan  Yokohama City University School of Medicine, Assistant, 医学部, 助手 (20326036)
YAMANAKA Shoji  Yokohama City University Hospital, Lecturer, 医学部附属病院, 講師 (80264604)
NAGASHIMA Yoji  Yokohama City University School of Medicine, Associate Professor, 医学部, 助教授 (10217995)
NOZAWA Akinori  Yokohama City University Hospital, Lecturer, 医学部附属病院, 講師 (00228321)
Project Period (FY) 2002 – 2003
Project Status Completed (Fiscal Year 2003)
Budget Amount *help
¥2,200,000 (Direct Cost: ¥2,200,000)
Fiscal Year 2003: ¥1,100,000 (Direct Cost: ¥1,100,000)
Fiscal Year 2002: ¥1,100,000 (Direct Cost: ¥1,100,000)
KeywordsInterstitial pneumonia / Hermansky-Pudlak syndrome / Type II pneumocyte / ep mouse / beige mouse / surfactant / ヘルマンスキーパドラック症候群 / NO / TNF-α / ヘルマンスキー・パドラック症候群
Research Abstract

We recently reported the constant presence of characteristic foamy swelling/degeneration (giant lamellar body degeneration ; GLBD) of type II pneumocytes in the lungs affected by Hermansky-Pudlak syndrome (HPS)-associated interstitial pneumonia (HPSIP), proposing the hypothesis that GLBD may be the triggering factor in the development of HPSIP (Virchows Arch, 2000). We now investigated the lung pathology of pale ear (ep) mouse, a mouse model of HPS1, and of beige (bg) mouse, a mouse model of Chediak-Higashi syndrome (CHS) with a special reference to GLBD and associated pathologic changes. GLBD was found both in ep and bg mice soon after birth, increasingin severity as mice grew older. Younger mice showed only GLBD with no evidence of interstitial change. Aged bg mice (22-24 months) showed the most prominent GLBD, 3 of the 8 cases showing associated lymphocytic infiltration and slight fibrosis of the collapsed alveolar septa as well as intraalveolar macrophage infiltration. Aged ep mice (24 months) with less severe GLBD than that of bg mice of comparative ages also showed a slight tendency to interstitial inflammation but no fibrosis. GLBs in these mice pneumocytes were immunoreactive for surfactant protein B and composed of lamellar structures ultrastructurally, almost identical to human GLBs. The results of the present study supports the hypothesis that GLBD may be playing an important role in the development of HPSIP. Ep and bg mice, especially the latter, may be a useful mouse model of HPSIP.

Report

(3 results)
  • 2003 Annual Research Report   Final Research Report Summary
  • 2002 Annual Research Report
  • Research Products

    (4 results)

All Other

All Publications (4 results)

  • [Publications] 中谷行雄, 中村宣生, 山中正二, 宮城洋平, 長嶋洋治, 他: "Hermansy-Pudlak症候群"日本胸部臨床. 62. S210-S219 (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Nakatani, Y et al.: "Hermansky-Pudlak syndrome."Nippon Kyobu Rinsho. 62(11). S210-S219 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] 中谷行雄, 中村宣生, 山中 正二, 宮城洋平, 長嶋洋治, 唐 小燕, 他: "特発性間質性肺炎とその周辺「Hennansky-Pudlak症候群」"日本胸部臨床. 62巻11号. S210-S219 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] 中谷行雄, 唐 小燕, 山中 正二, 長嶋洋治, 野沢昭典, 他: "特発性間質性肺炎とその周辺「Hermansky-Pudlak症候群」"日本胸部臨床. 増刊号(発表予定). (2003)

    • Related Report
      2002 Annual Research Report

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Published: 2002-04-01   Modified: 2016-04-21  

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