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Approach to cerebral small vessel disease: lesson from CARASIL.

Research Project

Project/Area Number 25293200
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypePartial Multi-year Fund
Section一般
Research Field Neurology
Research InstitutionNiigata University

Principal Investigator

Onodera Osamu  新潟大学, 脳研究所, 教授 (20303167)

Co-Investigator(Kenkyū-buntansha) SATO Toshiya  北里大学, 医学部, 教授 (90359703)
TOYOSHIMA Yasuko  新潟大学, 脳研究所, 准教授 (20334675)
KOYAMA Akihide  新潟大学, 研究推進機構, 助教 (90622209)
Project Period (FY) 2013-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥18,720,000 (Direct Cost: ¥14,400,000、Indirect Cost: ¥4,320,000)
Fiscal Year 2015: ¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2014: ¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2013: ¥9,360,000 (Direct Cost: ¥7,200,000、Indirect Cost: ¥2,160,000)
Keywords脳血管 / 神経病態 / 脳循環 / 神経病帯 / 脳小血管
Outline of Final Research Achievements

Cerebral small-vessel disease (CSVD) is a neurological disorder involving white matter lesions. CSVD is frequently observed in an elderly population and causes cognitive impairment and motor dysfunction. However little is known about a molecular pathogenesis for CSVD. Cerebral autosomal-recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL), an autosomal-recessive inherited cerebral small vessel disease (CSVD), involves severe leukoaraiosis, multiple lacunar infarcts, early-onset alopecia, and spondylosis deformans without hypertension. High-temperature requirement serine peptidase A1 (HTRA1) gene mutations cause CARASIL by decreasing HTRA1 protease activity. We have investigated the HTRA1 deficit mouse. We found that these mice showed mural cell and internal elastic membrane degeneration. We found fibronectin and protein X accumulates at the internal membrane. The pathological findings of the mouse resemble those of CARASIL as well as sporadic CSVD.

Report

(4 results)
  • 2015 Annual Research Report   Final Research Report ( PDF )
  • 2014 Annual Research Report
  • 2013 Annual Research Report
  • Research Products

    (12 results)

All 2016 2015 2014 2013

All Journal Article (7 results) (of which Peer Reviewed: 6 results,  Acknowledgement Compliant: 4 results) Presentation (5 results) (of which Int'l Joint Research: 2 results,  Invited: 1 results)

  • [Journal Article] Distinct molecular mechanisms of HTRA1 mutants in manifesting heterozygotes with CARASIL2016

    • Author(s)
      Nozaki H, Kato T, Nihonmatsu M, Saito Y, Mizuta I, Noda T, Koike R, Miyazaki K, Kaito M, Ito S, Makino M, Koyama A, Shiga A, Uemura M, Sekine Y, Murakami A, Moritani S, Hara K, Yokoseki A, Kuwano R, Endo N, Momotsu T, Yoshida M, Nishizawa M, Mizuno T, Onodera O.
    • Journal Title

      Neurology

      Volume: 未定

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Characteristic features and progression of abnormalities on MRI for CARASIL.2015

    • Author(s)
      Nozaki H, Sekine Y, Fukutake T, Nishimoto Y, Shimoe Y, Shirata A, Yanagawa S, Hirayama M, Tamura M, Nishizawa M, Onodera O.
    • Journal Title

      Neurology.

      Volume: 85 Issue: 5 Pages: 459-463

    • DOI

      10.1212/wnl.0000000000001803

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Characteristic features and progression of abnormalities on MRI for CARASIL2015

    • Author(s)
      Hiroaki Nozaki, Yumi Sekine, Toshio Fukutake, Yoshinori Nishimoto, Yutaka Shimoe, Akiko Shirata, Sohei Yanagawa, Mikio Hirayama, Masato Tamura, Masatoyo Nishizawa, and Osamu Onodera
    • Journal Title

      Neurology

      Volume: 未定

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Emerging molecular mechanism for cerebral small vessel disease: Lessons from hereditary small vessel disease2015

    • Author(s)
      Onodera, O., Sekine, Y., Kato, T., Koyama, A., Nozaki, H. and Nishizawa, M
    • Journal Title

      Neurology and Clinical Neuroscience

      Volume: 1 Issue: 1 Pages: 7-13

    • DOI

      10.1111/ncn3.134

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Features of cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy.2014

    • Author(s)
      Nozaki H, Nishizawa M, Onodera O.
    • Journal Title

      Stroke.

      Volume: 45 Issue: 11 Pages: 3447-3453

    • DOI

      10.1161/strokeaha.114.004236

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Haploinsufficiency of CSF-1R and clinicopathological characterization in patients with HDLS.2014

    • Author(s)
      Konno T, Tada M, Tada M, Koyama A, Nozaki H, Harigaya Y, Nishimiya J, Matsunaga A, Yoshikura N, Ishihara K, Arakawa M, Isami A, Okazaki K, Yokoo H, Ihoh K, Yoneda M, Kawamura M, Inuzuka T, Takahashi H, Nishizawa M, Onodera O, Kakita A, Ikeuchi T
    • Journal Title

      Neurology

      Volume: 82 Issue: 2 Pages: 139-148

    • DOI

      10.1212/wnl.0000000000000046

    • Related Report
      2013 Annual Research Report
    • Peer Reviewed
  • [Journal Article] [Hereditary cerebral small-vessel disease].2013

    • Author(s)
      Nozaki H, Nishizawa M, Onodera O.
    • Journal Title

      Nihon Rinsho.

      Volume: 71 Pages: 545-554

    • NAID

      40017342931

    • Related Report
      2013 Annual Research Report
  • [Presentation] Dominant Negative HTRA1 Mutations in Cerebral Small Vessel Disease.2015

    • Author(s)
      Nozaki H, Kato T, Nihonmatsu M, Saito Y, Mizuta I, Noda T, Koike R, Miyazaki K, Kaito M, Ito S, Makino M, Koyama A, Shiga A, Uemura M, Sekine Y, Murakami A, Moritani S, Hara K, Yokoseki A, Kuwano R, Endo N, Momotsu T, Yoshida M, Nishizawa M, Mizuno T, Onodera O.
    • Organizer
      9th International Congress on Vascular Dementia; ICVD 2015
    • Place of Presentation
      Ljubljana, Slovenija
    • Year and Date
      2015-10-16
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Characteristic brain MRI features in HTRA1 mutation heterozygotes.2015

    • Author(s)
      Masahiro Uemura, Hiroaki Nozaki, Yumi Sekine, Ikuko Mizuta, Tomoko Noda, Kazuhide Miyazaki, Muichi Kaito, Yoshinori Nishimoto, Yutaka Shimoe, Akiko Shirata, Kiyomi Yamane, Sohei Yanagawa, Mikio Hirayama, Masato Tamura, Toshiki Mizuno, Masatoyo Nishizawa, and Osamu Onodera.
    • Organizer
      9th International Congress on Vascular Dementia; ICVD 2015
    • Place of Presentation
      Ljubljana, Slovenija
    • Year and Date
      2015-10-16
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Presentation] GFβ1過剰発現マウスでは脳小血管壁細胞の異常をきたす2014

    • Author(s)
      関根有美、加藤泰介、野崎洋明、廣川祥子、佐藤俊哉、志賀篤、笹岡敏邦、西澤正豊、小野寺理
    • Organizer
      第55回日本神経学会学術大会
    • Place of Presentation
      福岡市 福岡国際会議場
    • Year and Date
      2014-05-21 – 2014-05-24
    • Related Report
      2014 Annual Research Report
  • [Presentation] MRI Features of Cerebral Autosomal Recessive Arteriopathy with Subcortical Infarcts and Leukoencephalopathy.2013

    • Author(s)
      Hiroaki Nozaki, Yumi Sekine, Toshio Fukutake, Yoshinori Nishimoto, Mamoru Shibata, Akiko Shirata, Kiyomi Yamane, Yutaka Shimoe, Sohei Yanagawa, Mikio Hirayama, Imaharu Nakano, Norihiro Suzuki, Masatoyo Nishizawa, Osamu Onodera.
    • Organizer
      The 8th International Congress on Vascular Dementia
    • Place of Presentation
      アテネ ギリシャ
    • Related Report
      2013 Annual Research Report
  • [Presentation] An emerging molecular mechanism for the cerebral small vessel disease2013

    • Author(s)
      Osamu Onodera
    • Organizer
      54th Annual Meeting of the Japanese Society of Neurology
    • Place of Presentation
      Tokyo
    • Related Report
      2013 Annual Research Report
    • Invited

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Published: 2013-05-21   Modified: 2019-07-29  

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