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Establishment of genetic lipid storage disease with lysosomal acid lipase deficiency in rats

Research Project

Project/Area Number 63480144
Research Category

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

Allocation TypeSingle-year Grants
Research Field Experimental pathology
Research InstitutionKagoshima University

Principal Investigator

YOSHIDA Hiroki  Kagoshima University, Faculty of Medicine, Professor, 医学部, 教授 (90036476)

Co-Investigator(Kenkyū-buntansha) FUJIYOSHI Toshinobu  kagoshima University, Faculty of Medicine, Assistant Professor, 医学部, 助手 (50173480)
YOSHIDA Aichi  Kagoshima University, Faculty of Medicine, Associate Professor, 医学部, 助教授 (20036453)
Project Period (FY) 1988 – 1989
Project Status Completed (Fiscal Year 1989)
Budget Amount *help
¥3,500,000 (Direct Cost: ¥3,500,000)
Fiscal Year 1989: ¥1,000,000 (Direct Cost: ¥1,000,000)
Fiscal Year 1988: ¥2,500,000 (Direct Cost: ¥2,500,000)
KeywordsCholesterol Ester / Triglyceride / Acid Lipase / Rat / コレステロールエステル
Research Abstract

We established a new animal model of a genetic lipid storage disease analogous to human Wolman's disease. Affected Donryu rats, who inherited the disease in an autosomal recessive mode, manifested marked hepatosplenomegaly, lymph node enlargement, and thickened, dilated intestine. Morphologically, many characteristic foam cells Were observed in livers and spleens. No adrenal calcification could be found in affected rats. Biochemical studies on spleen and liver tissues showed massive accumulation of esterified cholesterol and triglycerides and deficiency of acid lipase for [^<14>C] -triolen, [^<14>C]-cholesterol oleate, and 4-methylumbelliferyl oleate.
This animal model could contribute greatly to the clarification of the physiological and pathological roles of lysosomal acid lipase in the metabolism of lipoproteins and cholesterol, and of the pathogeneisis of atherosclerosis.

Report

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

    (10 results)

All Other

All Publications (10 results)

  • [Publications] Hiroki Yoshida,et al.: "Genetic lipid storage disease with lysosomal acid lipase deficiency in rats." Laboratory Animal Science(印刷中). (1990)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Masaru Kuriyama,Hiroki Yoshida,et al.: "Lyosomal acid lipase deficiency in rats;Lipid analyses and lipase activities in livers and spleens." Biochemica et Biophysica Acta(投稿中).

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Hiroki Yoshida,Aichi Yoshida, et al.: "Lyosomal acid lipase deficiency in rats;Immunohistochemical and electron microscopic studies." Amirican Journal of Pathology(投稿予定).

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Hiroki Yoshida, et al: "Genetic lipid storage disease with lysosomal acid lipase deficiency in rats." Laboratory Animal Science 1990.

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Masaru Kuriyama, Hiroki Yoshida, et al.: "Lysosomal acid lipase deficiency in rats ; Lipids analyses and lipase activities in livers and spleens." Biochemica et Biophysica Acta.

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Hiroki Yoshida, Aichi Yoshida et al.: "Lyosomal acid lipase deficiency in rats : immunohistochemical and electron microscopic studies."

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1989 Final Research Report Summary
  • [Publications] Hiroki Yoshida,et al.: "Genetic lipid storage disease with lysosomal acid lipase deficiency in rats." Laboratory Animal Science. (1990)

    • Related Report
      1989 Annual Research Report
  • [Publications] Masaru Kuriyama,Hiroki Yoshida,el al.: "Lysosomal acid lipase deficiency in rats;Lipid analyses and lipases activities in livers and spleens." Biocemica et Biophysica Acta.

    • Related Report
      1989 Annual Research Report
  • [Publications] Hiroki Yoshida,Aichi Yoshida,et al.: "Lysosomal acid lipase dificiency in rats;Immunohisto-chemical and electron microscopic studies." American Journal of Pathology.

    • Related Report
      1989 Annual Research Report
  • [Publications] Hiroki Yoshida;Masaru Kuriyama;Aichi Yoshida;Akihiro Igata.: The American Journal of Pathology.

    • Related Report
      1988 Annual Research Report

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

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