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臨床化学的手法を基盤とする先天性胆汁酸代謝異常症の病態解析

Research Project

Project/Area Number 15K01809
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Biomolecular chemistry
Research InstitutionNihon University

Principal Investigator

飯田 隆  日本大学, 文理学部, 教授 (60060125)

Project Period (FY) 2015-04-01 – 2017-03-31
Project Status Discontinued (Fiscal Year 2016)
Budget Amount *help
¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000)
Fiscal Year 2017: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2016: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2015: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Keywords造血幹細胞 / 胆汁酸 / 二次胆汁酸 / 液体クロマトグラフータンデム質量分析 / IEBAM疾患 / NPDC疾患 / LC-MS/MS / 異常胆汁酸 / 多重抱合体
Outline of Annual Research Achievements

全ての血液細胞の源である造血幹細胞は、大人の体内では骨髄の内部で休眠状態にあるが、発生中の胎児では肝臓(胎児肝)で活発に増殖している。造血幹細胞はストレスに弱く、体外で増殖させるとすぐに機能が低下してしまうことが知られているため、胎児肝の中ではどうして増殖が可能であるのか謎のままであった。当研究グループは以前、造血幹細胞はタンパク質を正常に折りたたむ能力が低いため、体外培養すると異常なタンパクが蓄積して死んでしまうことを発見していた。今回、申請者らは胎児肝では食物の消化に使われる胆汁の主成分である“胆汁酸”が異常タンパク質の蓄積を防いでいることを明らかにした。胎児肝の胆汁酸が減少するマウスを解析したところ、増殖するはずの造血幹細胞が大幅に減少していることが判明した。これらの発見は、白血病治療などに対する骨髄・臍帯血移植技術の改良に繋がるだけでなく、妊娠期に胎児に起こる疾病の解明など貢献するものと期待される。

Report

(2 results)
  • 2016 Annual Research Report
  • 2015 Research-status Report
  • Research Products

    (11 results)

All 2017 2016 2015

All Journal Article (9 results) (of which Int'l Joint Research: 9 results,  Peer Reviewed: 9 results,  Open Access: 7 results,  Acknowledgement Compliant: 3 results) Presentation (2 results)

  • [Journal Article] 3,4-Seco-12α-hydroxy-5β-cholan-3,4,24-trioic acid, a novel secondary bile acid: isolation from the bile of the common ringtail possum (Preudocheirus peregrinus) and chemical synthesis.2017

    • Author(s)
      S.Sekiguchi, K.Namegawa, N.Nakane, R.Satoh, K.Omura, L.R.Hagey, A.F.Hofmann, T.Iida.
    • Journal Title

      J. Pharmacogn. Nat. Prod.

      Volume: 3 Issue: 01 Pages: 1000131-1000131

    • DOI

      10.4172/2472-0992.1000131

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research / Acknowledgement Compliant
  • [Journal Article] 2.Novel, major 2α- and 2β-hydroxy bile alcohols and bile acids in the bile of Arapaima gigas, a large South American river fish.2016

    • Author(s)
      Sato Née Okihara R, Saito T, Ogata H, Nakane N, Namegawa K, Sekiguchi S, Omura K, Kurabuchi S, Mitamura K, Ikegawa S, Raines J, Hagey LR, Hofmann AF, Iida T.
    • Journal Title

      Steroids

      Volume: 107 Pages: 112-120

    • DOI

      10.1016/j.steroids.2016.01.001

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Bile Acids Protect Expanding Hematopoietic Stem Cells from Unfolded Protein Stress in Fetal Liver2016

    • Author(s)
      V.Sigurdsson, H.Takei, S.Sobeleva, V.Radulovic, R.Galeev, K.Siva, L.M.F.Leeb-Lundberg, T.Iida, H.Nittono, K.Miharada.
    • Journal Title

      Cell Stem Cell

      Volume: 18 Issue: 4 Pages: 522-532

    • DOI

      10.1016/j.stem.2016.01.002

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Two Major Bile Acids in the Hornbills, (24R,25S)-3α,7α,24-Trihydroxy-5β-cholestan-27-oyl Taurine and Its 12α-Hydroxy Derivative2016

    • Author(s)
      R.Satoh, H.Ogata, T.Saito, B.Zhou, K.Omura, S.Kurabuchi, K.Mitamura, S.Ikegawa, L.R.Hagey, A.F.Hofmann, T.Iida.
    • Journal Title

      Lipids

      Volume: 51 Issue: 6 Pages: 757-768

    • DOI

      10.1007/s11745-016-4150-0

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research / Acknowledgement Compliant
  • [Journal Article] Identification of novel bile acids as biomarkers for the early diagnosis of Niemann‐Pick C disease2016

    • Author(s)
      F.Mazzacuva, P.Mills, K.Mills, S.Camuzeaux, P.Gissen, E-R Nicoli, C.Wassif, D.Vruchte, F.D.Porter, M.Maekawa, N.Mano, T.Iida, F.Platt, P.T.Clayton.
    • Journal Title

      FEBS Letters

      Volume: 590 Issue: 11 Pages: 1651-1662

    • DOI

      10.1002/1873-3468.12196

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Rifaximin Exerts Beneficial Effects Independent of its Ability to Alter Microbiota Composition2016

    • Author(s)
      D.J.Kang, G.Kakiyama, N.S.Betrapally, J.Herzog, H.Nittono, P.B.Hylemon, H.Zhou, I.Carroll, J.Yang, P.M.Gillevet, C.Jiao, H.Takei, W.H.Pandak, T.Iida, D.M.Heuman, S.Fan, O.Fiehn, T.Kurosawa, M.Sikaroodi, R.B.Sartor, J.S.Bajaj.
    • Journal Title

      Clinical and Translational Gastroenterology

      Volume: 7 Issue: 8 Pages: e187-e187

    • DOI

      10.1038/ctg.2016.44

    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Chemical Synthesis of Uncommon Natural Bile Acids: The 9α-Hydroxy Derivatives of Chenodeoxycholic and Lithocholic Acids2016

    • Author(s)
      T.Iida, K.Namegawa, N.Nakane, K.Iida, A.F.Hofmann, K.Omura.
    • Journal Title

      Chemical and Pharmaceutical Bulletin

      Volume: 64 Issue: 9 Pages: 1397-1402

    • DOI

      10.1248/cpb.c16-00247

    • NAID

      130005261594

    • ISSN
      0009-2363, 1347-5223
    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research / Acknowledgement Compliant
  • [Journal Article] Identification of Two Sulfated Cholesterol Metabolites Found in the Urine of a Patient with Niemann–Pick Disease Type C as Novel Candidate Diagnostic Markers2016

    • Author(s)
      M.Maekawa, K.Omura, S.Sekiguchi, T.Iida, D.Saigusa, H.Yamaguchi, N.Mano.
    • Journal Title

      Mass Spectrometry

      Volume: 5 Issue: 2 Pages: S0053-S0053

    • DOI

      10.5702/massspectrometry.S0053

    • NAID

      130005285752

    • ISSN
      2186-5116, 2187-137X
    • Related Report
      2016 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Focused metabolomics using liquid chromatography/electrospray ionization tandem mass spectrometry for analysis of urinary conjugated cholesterol metabolites from patients with Niemann-Pick disease type C and 3β-hydroxysteroid dehydrogenase deficiency2015

    • Author(s)
      Masamitsu Maekawa, Miki Shimada, Kousaku Ohno, Masami Togawa, Hiroshi Nittono, Takashi Iida, Alan F Hofmann, Junichi Goto, Hiroaki Yamaguchi, and Nariyasu Mano
    • Journal Title

      Annals of Clinical Biochemistry

      Volume: 52(5) Issue: 5 Pages: 576-587

    • DOI

      10.1177/0004563214568871

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Presentation] 低出生体重児を含む小児におけるUDCAのN-アセチルグルコサミン抱合能について2016

    • Author(s)
      武井一、入戸野博、飯田隆、村井毅、黒澤隆夫
    • Organizer
      第38回 胆汁酸研究会
    • Place of Presentation
      久留米シティプラザ(福岡県久留米市)
    • Year and Date
      2016-11-26
    • Related Report
      2016 Annual Research Report
  • [Presentation] 先天性胆汁酸生合成異常症ハイリスク・スクリーニングによるNiemann-Pick 病C型の早期発見について2016

    • Author(s)
      成高中之、鈴木光幸、武井一、水落建輝、木村昭彦、塩田光隆、梅津守一郎、十河剛、乾あやの、前川正充、眞野成康、入戸野博、飯田隆、清水俊明
    • Organizer
      第38回 胆汁酸研究会
    • Place of Presentation
      久留米シティプラザ(福岡県久留米市)
    • Year and Date
      2016-11-26
    • Related Report
      2016 Annual Research Report

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Published: 2015-04-16   Modified: 2018-01-16  

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