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Pathogenesis of Pompe's Disease

Research Project

Project/Area Number 01570204
Research Category

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

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

Principal Investigator

IWAMASA Teruo  Ryukyu University, Pathology, Professor., 医学部, 教授 (10110842)

Co-Investigator(Kenkyū-buntansha) NAKASHIMA Yasutsugu  Ryukyu University, Biochemistry, Associate Professor, 医学部, 助教授 (90180281)
UTSUMI Yasufumi  Ryukyu University, Pathology, Assistant, 医学部, 助手 (90203562)
大下 健幸  徳島大学, 酵素研究センター, 助手 (30185243)
Project Period (FY) 1989 – 1990
Project Status Completed (Fiscal Year 1990)
Budget Amount *help
¥1,800,000 (Direct Cost: ¥1,800,000)
Fiscal Year 1990: ¥600,000 (Direct Cost: ¥600,000)
Fiscal Year 1989: ¥1,200,000 (Direct Cost: ¥1,200,000)
KeywordsLysosomal acid alpha-glucosidase / Pompe's disease / 酸性α-グルコシダ-ゼ / 酸性α-グルコシダ-ゼcDNA
Research Abstract

We purified acid alpha-glucosidase from human placenta and pig liver. The purification procedures were followed essentially as described previously. Recently we found that the enzyme showed the heterogeneity in its affinity to Sephacryl s-200 gel. The enzyme was separated into two major components (S1 and S2). Each component was further separated into two (76 KDa and 67 KDa components) by SDS-PAGE and also chromatofocusing. The tissue distribution of these four components (76 KDa and 67 KDa of S1 and S2 components) was investigated. The liver, heart and kidney enzymes were consisted of these four components. However, the macrophage enzyme contained only S1 76 KDa component. The 4-methylumbelliferyl alpha-glucosidase activities of these four components were all inhibited competitively by glycogen, maltose, isomaltose and turanose, which suggests that the acid alpha-glucosidase has a single substrate binding site which is common to those substrates. According to the amino acid sequence a … More nalysis, the four components were considered to be the same gene product. Sugar chain analysis of these four components and CRM (immunologically cross-reactive material which reacts with anti acid alpha-glucosidase) from Pompe's disease was performed by the lectin-nitrocellulose sheet method. The S1 and S2 components and CRM contained the mannose. Con A staining patterns before and after digestion with Endo H, Endo F and PNGase indicated that there may be small amounts of other sugar chain different from mannose-rich N-linked chain. In addition, CRM from Pompe's disease contains small amounts of sialic acid in its sugar chains. These experiments were also carried out using Pompe's disease fibroblasts which were transfected with SV 40 large T DNA. cDNA and mRNA from Pompe's disease fibroblasts are now investigating.
From a cytopathological view point, lysosomal storage disease may be classified into two groups : A and B. In the disease of A group, the storage substances are mainly observed in the parenchymal cells. In the Pompe's disease, large amounts of glycogen were stored in the hepatocytes and muscle. The acid alpha-glucosidase localized mainly in the parenchymal cells such as hepatocytes and muscle cells. On the other hand, in the diseases of group B, the storage substances were found in the macrophages as in the case of Hurler disease. We purified alpha-L-iduronidase from human placenta and pig liver. alpha-L-Iduronidase was demonstrated immunohistochemically in the macrophages. We are now investigating the lysosomal diseases, group A and B, pathologically, biochemically and genetically. Less

Report

(3 results)
  • 1990 Annual Research Report   Final Research Report Summary
  • 1989 Annual Research Report
  • Research Products

    (25 results)

All Other

All Publications (25 results)

  • [Publications] Kuriyama M,Kohriyama T.,Iwamasa T.et al.: "Lymphocyte αーglucosidase in lateーonset glycogenosis type II" Arch.Neurol. 46. 460-462 (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] Ohshita T.,Sakuda H.,Nakasone S.and Iwamasa T.: "Purification,characterization and subcellular localization of pig liver αーLーiduronidase" Eur.J.Biochem. 179. 201-207 (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] Iwamasa T.,Sakuda H.,Nakasone S.and Ohshita T.: "Pathologic studies of lysesomal storage disease" Acta Histochem Cytochem.23. 65-80 (1990)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] Sakuda H.,Kusada A.,Ohshita T.and Iwamasa T.: "Tissue and cellular distribution of αーLーiduronidase in pig" J.Histochem.Cytochem.38. 785-792 (1990)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] Nakasone S.,Ohshita T.and Iwamasa T.: "Heterogeneity of pig lysosomal acid αーglucosidase:Affinity to Sephacryl Sー200 gel and tissue distribution" Biochem J.(1991)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] 岩政 輝男: "筋病理学 檜沢 埜中 小沢編" 文光堂, 18-480 (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] 岩政 輝男: "現代病理学大系 11a" 中山書店, 9-409 (1990)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] Kuriyama M, Kohriyama T, Iwamasa T, et al.: "Lymphocytes alpha-glucosidase in late-onset glycogenosis type II" Arch. Neurol.46. 460-462 (1989)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] Ohshita T, Sakuda H, Nakasone S, and Iwamasa T.: "Purification, characterization and subcellular localization of pig liver alpha-L-iduronidase" Eur. J. Biochem.179. 201-207 (1989)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] Iwamasa T, Sakuda H, Nakasone S, and Ohshita T.: "Pathologic studies of lysosomal storage disease" Acta Histochem. Cytochem.23. 65-80 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] Sakuda H, Kusaba A, Ohshita T, and Iwamasa T.: "Tissue and cellular distribution of alpha-L-iduronidase" J. Histochem. Cytochem.38. 785-792 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] Nakasone S, Ohshita T, and Iwamasa T.: "Heterogeneity of pig lysosomal acid alpha-glucosidase : Affinity to Sephacryl S-200 gel and tissue distribution" Biochem. J.(1991)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1990 Final Research Report Summary
  • [Publications] Kuriyama M,Kohriyama T.,Iwamasa T.et al.: "Lymphocyte αーglucosidase in lateーonset glycogenosis type II" Arch.Neurol. 46. 460-462 (1989)

    • Related Report
      1990 Annual Research Report
  • [Publications] Ohshita T.,Sakuda H.,Nakasone S.and Iwamasa T.: "Purification,characterization and subcellular localizaton of pig liver αーLーiduronidase" Eur.J.Biochem. 179. 201-207 (1989)

    • Related Report
      1990 Annual Research Report
  • [Publications] Iwamasa T.,Sakuda H.,Nakasone S.and Ohshita T.: "Pathologic studies of lysosomal storage disease" Acta Histochem.Cytochem.23. 65-80 (1990)

    • Related Report
      1990 Annual Research Report
  • [Publications] Sakuda H.,Kusaba A.,Ohshita T.and Iwamasa T.: "Tissue and cellular distribution of αーLーiduronidase in pig" J.Histochem.Cytochem.38. 785-792 (1990)

    • Related Report
      1990 Annual Research Report
  • [Publications] Nakasone S.,Ohshita T.and Iwamasa T.: "Heterogeneity of pig lysosomal acid αーglucosidase: Affinity to Sephacryl Sー200 gel and tissue distribution" Biochem J.(1991)

    • Related Report
      1990 Annual Research Report
  • [Publications] 岩政 輝男: "筋病理学 檜沢.埜中.小沢編" 文光堂, 18/480 (1989)

    • Related Report
      1990 Annual Research Report
  • [Publications] 岩政 輝男: "現代病理学大系11a" 中山書店, 9/409 (1990)

    • Related Report
      1990 Annual Research Report
  • [Publications] M.Kuriyama,T.Kohriyama,T.Iwamasa,R.Hiwatari,M.Osame and A.Igata: "Lymphocyte α-glucosidase in late-onset glycogenosis type II" Archives of Neurology. 46. 460-463 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] 大下健幸,仲宗根進,岩政輝男: "ヒトおよびブタ酸性α-グルコシダ-ゼの不均一性について" 生化学. 61. 1050 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] 屋上麻利子,瀧尾壙士,千谷昇,岩政輝男: "酸性α-グルコシダ-ゼの4分子種について" 生化学. 61. 1050 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] T.Ohshita,H.Sakuda,S.Nakasone,T.Iwamasa: "Purification,characterization and subcellular distribution of pig liver α-L-iduronidase" Eur.J.Biochem.179. 201-207 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] 岩政輝男: "筋病理学、(桧澤、埜中、小川編)" 文光堂, 18 -465 (1989)

    • Related Report
      1989 Annual Research Report
  • [Publications] 岩政輝男: "病理学、(遠城寺編)" 医学書院, 35 -869 (1989)

    • Related Report
      1989 Annual Research Report

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Published: 1989-04-01   Modified: 2016-04-21  

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