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Analysis of the role of sulfur amino acids metabolism in renal proximal tubules using mice lacking cysteine-producing enzymes

Research Project

Project/Area Number 22790219
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeSingle-year Grants
Research Field General physiology
Research InstitutionAkita University (2011)
Keio University (2010)

Principal Investigator

AKAHOSHI Noriyuki  秋田大学, 大学院・医学系研究科, 助教 (70534551)

Project Period (FY) 2010 – 2011
Project Status Completed (Fiscal Year 2011)
Budget Amount *help
¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2011: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2010: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Keywords含硫アミノ酸 / Cystathionine β-synthase (CBS) / Cystathionine γ-lyase (CSEまたはCTH) / 硫化水素 / ホモシスチン尿症 / 妊娠高血圧腎症 / Cystathionine β-synthase / Cystathionine γ-lyase / アミノ酸トランスポーター / Cystationine β-synthase / Cystationine γ-lyase
Research Abstract

Cystathionine β-synthase (CBS) and cystathionine γ-lyase (CSE) are the transsulfuration enzymes that are involved in sulfur amino acids metabolism. CBS-deficient patients show severe clinical symptoms, while CSE-deficient persons are considered to be free of any clinical manifestations. Although both enzymes are primarily expressed in the liver and the kidney of mice, their roles in the kidney remain to be elucidated. In CBS-deficient mice, the urinary excretion of toxic methionine was much lower efficiency, while the cystathionine was efficiently excreted into the urine in CSE-deficient mice. The efficiency of excretion is considered to be related to the difference of symptoms. Pregnancy-induced hypertension and proteinuria was developed in normal-appearing CSE-deficient mice. H2S that has vasorelaxant activity is known to be produced by CBS and CSE, and its distribution was overlapped with both enzymes in kidney.

Report

(3 results)
  • 2011 Annual Research Report   Final Research Report ( PDF )
  • 2010 Annual Research Report
  • Research Products

    (10 results)

All 2012 2011 2010

All Journal Article (8 results) (of which Peer Reviewed: 8 results) Presentation (2 results)

  • [Journal Article] Methionine excess in diet induces acute lethal hepatitis in mice lacking cystathionine γ-lyase, an animal model of cystathioninuria2012

    • Author(s)
      Yamada H, Akahoshi N, Kamata S, Hagiya Y, Hishiki T, Nagahata Y, Matsuura T, Takano N, Mori M, Ishizaki Y, Izumi T, Kumagai Y, Kasahara T, Suematsu M, Ishii I
    • Journal Title

      Free Radic Biol Med

      Volume: 52 Pages: 1716-1726

    • Related Report
      2011 Final Research Report
    • Peer Reviewed
  • [Journal Article] TOF-SIMS imaging of halide/thiocyanate anions and hydrogen sulfide in mouse kidney sections using silver-deposited plate2012

    • Author(s)
      Akahoshi N., Ishizaki I., Naya M., Maekawa T., Yamozoe S., Horiuchi T., Kajimura M., Ohashi Y., Suematsu M., Ishii I.
    • Journal Title

      Anal Bioanal Chem

      Volume: 402 Issue: 5 Pages: 1859-1864

    • DOI

      10.1007/s00216-011-5647-2

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Methionine excess in diet induces acute lethal hepatitis in mice lacking cystathionine gamma-lyase, an animal model of cystathioninuria.2012

    • Author(s)
      Yamada H, Akahoshi N, Kamata S, Hagiya Y, Hishiki T, Nagahata Y, Matsuura T, Takano N, Mori M, Ishizaki Y, Izumi T, Kumagai Y, Kasahara T, Suematsu M, Ishii I
    • Journal Title

      Free Radic Biol Med

      Volume: 52 Issue: 9 Pages: 1716-1726

    • DOI

      10.1016/j.freeradbiomed.2012.02.033

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] TOF-SIMS imaging of halide/thiocyanate anions and hydrogen sulfide in mouse kidney sections using silver-deposited plates2011

    • Author(s)
      Akahoshi N, Ishizaki I, Naya M, Maekawa T, Yamazoe S, Horiuchi T, Kajimura M, Ohashi Y, Suematsu M, Ishii I
    • Journal Title

      Anal. Bioanal. Chem

      Volume: 402 Pages: 1859-1864

    • Related Report
      2011 Final Research Report
    • Peer Reviewed
  • [Journal Article] Triacylglycerol/phospholipid molecular species profiling of fatty livers and regenerated non-fatty livers in cystathionine beta-synthase-deficient mice, an animal model for homocysteinemia/homocystinuria2011

    • Author(s)
      Ikeda K, Kubo A, Akahoshi N, Yamada H, Miura N, Hishiki T, Nagahata Y, Matsuura T, Suematsu M, Taguchi R, Ishii I
    • Journal Title

      Anal. Bioanal. Chem

      Volume: 400 Pages: 1853-1863

    • Related Report
      2011 Final Research Report
    • Peer Reviewed
  • [Journal Article] Triacylglycerol/phospholipid molecular species profiling of fatty livers and regenerated non-fatty livers in cystathionine beta-synthase-deficient mice, an animal model for homocysteinemia/homocystinuria.2011

    • Author(s)
      Ikeda K, Kubo A, Akahoshi N, Yamada H, Miura N, Hishiki T, Nagahata Y, Matsuura T, Suematsu M, Taguchi R, Ishii I
    • Journal Title

      Analytical and Bioanalytical Chemistry.

      Volume: (Epub) Pages: 1-10

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Cystathionine gamma-Lyase-deficient mice require dietary cysteine to protect against acute lethal myopathy and oxidative injury2010

    • Author(s)
      Ishii I, Akahoshi N, Yamada H, Nakano S, Izumi T, Suematsu M
    • Journal Title

      J. Biol. Chem

      Volume: 285 Pages: 26358-26368

    • Related Report
      2011 Final Research Report
    • Peer Reviewed
  • [Journal Article] Cystathionine gamma-Lyase-deficient mice require dietary cysteine to protect against acute lethal myopathy and oxidative injury.2010

    • Author(s)
      Ishii I, Akahoshi N, Yamada H, Nakano S, Izumi T, Suematsu M
    • Journal Title

      Journal of Biological Chemistry.

      Volume: 285 Pages: 26358-26368

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Presentation] 高ホモシステイン血症マウスにおける妊娠高血圧症と蛋白尿2010

    • Author(s)
      赤星軌征, 末松誠, 石井功
    • Organizer
      第83回日本生化学会
    • Place of Presentation
      神戸
    • Year and Date
      2010-12-07
    • Related Report
      2010 Annual Research Report
  • [Presentation] Pregnancy-induced hypertension and proteinuria in hyperhomocysteinemic mice2010

    • Author(s)
      Akahoshi N, Suematsu M, Ishii I
    • Organizer
      日本生化学会大会
    • Place of Presentation
      神戸
    • Related Report
      2011 Final Research Report

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Published: 2010-08-23   Modified: 2016-04-21  

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