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Partial Overlaping between Protective Protein and Phospholipid Transfer Protein Genes

Research Project

Project/Area Number 09672316
Research Category

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

Allocation TypeSingle-year Grants
Section一般
Research Field Human genetics
Research InstitutionThe Tokyo Metropolitan Institute of Medical Science

Principal Investigator

SHIMMOTO Michie  The Tokyo Metropolitan Institute of Medical Science, Clinical Genetics Researche, 臨床遺伝学研究部門, 研究員 (20216237)

Project Period (FY) 1997 – 1998
Project Status Completed (Fiscal Year 1998)
Budget Amount *help
¥3,000,000 (Direct Cost: ¥3,000,000)
Fiscal Year 1998: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 1997: ¥2,200,000 (Direct Cost: ¥2,200,000)
Keywordsprotective protein / phospholipid transfer protein / gene overlap / galactosialidosis
Research Abstract

Protective protein (PP) is a multi functional glycoprotein which regulates the expression of b-galactosidase and neuraminidase, by stabilizing the former and by activating the letter, through the formation of multienzymic complex in lysosomes. Phospholipid transfer protein (PLTP) promotes the exchange and transfer of phospholipids. PLTP also has the ability to facilitate modulation of high density lipoprotein size and composition. It has been reported that genes encording both proteins are partially overlapping by 58 nucleotides at the parts of 3'-untranslated end sequences of their mRNAs on the complementary strand of Human genomic DNA.
In this study, we screened a bacterial artificial chromosome (BAC) library of mouse DNA by means of PCR-based analysis, and obtained a positive clone. The clone contained the entire mouse PP and PLTP genes. We determined the exon-intron organizations of the both genes by PCR amplifications using DNA of the clone as a template, and subsequent sequencing analysis of the amplified DNA fragments. The mouse PP gene spans 6.5kb and comprises 15 exons, and the mouse PLTP gene spans 18kb and comprises 16 exons. Compared with those of the human genes, the exon-intron organizations of the mouse PP and PLTP genes were well conserved. We Confirmed that 66nt sequence corresponding to the 3'-untranslated ends of mRNAs of both genes overlapped on the complementary strand of DNA.

Report

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

    (13 results)

All Other

All Publications (13 results)

  • [Publications] Shimmoto, M.: "Generation and Characterization of Transgenic Mice Expressing a Human α-Galactosidase with an R301Q Substitution Causing a Variant Form of Fabry Disease"FEBS Letter. 417. 89-91 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Takiyama, N.: "Molecular form and subcellular distribution of acid β-galactosidase in fibroblasts from patients with Morquio B disease and galactosialidosis"Brain Dev.. 19. 126-130 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Itoh, K.: "Protective protein/cathepsin A loss in cultured cells derived from an early-infantile form of galactosialidosis patients homozygous for the A1184→G transition (Y395C mutation)"Biochem.Biophys.Res.Commun. 247. 12-17 (1998)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Itoh, K.: "Stabilizing effect of lysosomal β-galactosidase on the catalytic activity of protective protein/cathepsin A secreted by human platelets"Biochem.Biophys.Res.Commun. 253. 228-234 (1998)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Takiyama, N., Itoh, K., Shimmoto, M., Nishimoto, M., Inui, K., Sakuraba, H.: "Molecular form and subcelluar distribution of acid β-galactosidase in fibroblasts from patients with Morquio B disease and galactosialidosis."Brain Dev.. 10. 126-130 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Shimmoto, M., Kase, R., Itoh, K., Utsumi, K., Ishii, S., Taya, C., Yonekawa, H., Sakuraba, H.: "Generation and characterizatiuon of transgenic mouse expressing a human mutant α-galactosidase with an R301Q substitution causing a variant form of Fabry disease."FEBS Lett.. 417. 89-91 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Itoh, K., Shimmoto, M., Utsumi, K., Mizoguchi, N., Miharu, N., Ohama, K., Sakuraba, H.: "Protective protein/cathepsin A loss in cultured cells-derived from an early-infantile form of galactosialidosis patients homozygous for the A1184-G transition (Y395C mutation)."Biochem. Biophys. Res. commun.. 247. 12-17 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Itoh, K., Naganawa, Y., Kamei, S., Shimmoto, M., Sakuraba, H.: "Stabilizing effect of lysosomal β-galactosidase on the catalytic activety of protective protein/cathepsin A secreted by human platelets"Biochem. Biophys. Res. Commun.. 253. 228-234 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Shimmoto,M.: "Generation and Characterization of Transgenic Mice Expressing a Human α-Galactosidase with an R301Q Substitution Cauzing a Variant Form of Fabry Disease" FEBS Letter. 417. 89-91 (1997)

    • Related Report
      1998 Annual Research Report
  • [Publications] Takiyama,N: "Molecular form and subcellular distribution of acid β-galactosidase in fibroblasts from patients with Morquio B disease and garactosialidosis" Brain Dev.19. 126-130 (1998)

    • Related Report
      1998 Annual Research Report
  • [Publications] Itoh,K.: "Protective protein/cathepsin A loss in cultured cells derived from an early-infantile form of galactosialidosis patients homezygous for A1184-G transition (Y595C matation)" Biochem.Biophys.Res.Commun.247. 12-17 (1998)

    • Related Report
      1998 Annual Research Report
  • [Publications] Itoh,K.: "Stabilizing effect of lysosomal β-galactosidase on the catalytic activity of protective protein/cathepsin A secreted by human platelets." Biochem.Biophys.Res.Commun. 253. 228-234 (1998)

    • Related Report
      1998 Annual Research Report
  • [Publications] Shimmoto,M.: "Generation and Characterization of Transgenic Mice Expressing a Human Mutant α-Galactosidase with an R301Q Substitution Causing a Variant Form of Fabry Disease." FEBS Letters. 417. 89-91 (1997)

    • Related Report
      1997 Annual Research Report

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

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