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Disease specific therapy of polycystic kidney disease based on its pathophysiology considering the ubiquitin-proteasome proteolysis mechanism

Research Project

Project/Area Number 23591580
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Pediatrics
Research InstitutionWakayama Medical University

Principal Investigator

NAKANISHI Koichi  和歌山県立医科大学, 医学部, 講師 (50336880)

Co-Investigator(Kenkyū-buntansha) YOSHIKAWA Norishige  和歌山県立医科大学, 医学部, 教授 (10158412)
Project Period (FY) 2011 – 2013
Project Status Completed (Fiscal Year 2013)
Budget Amount *help
¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
Fiscal Year 2013: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2012: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2011: ¥2,340,000 (Direct Cost: ¥1,800,000、Indirect Cost: ¥540,000)
Keywords多発性嚢胞腎 / CPKマウス / ユビキチン・プロテアソーム / 上皮間葉移行(EMT) / 疾患特異的治療
Research Abstract

Polycystic kidney disease is an inherited disease, which is the most frequent in our country, and is one of main causes of chronic renal failure. There are two types of polycystic kidney disease, autosomal dominant (ADPKD) and autosomal recessive (ARPKD).
In this study, we elucidated ubiquitin-proteasome proteolysis mechanism in multiple pathophysiology of polycystic kidney disease, and aimed to confirm the effect with modification of ubiquitin-proteasome proteolysis using the model animal for disease specific therapy development based on the pathophysiology of polycystic kidney disease to adapt it to human. We focused on ubiquitin E3 ligase family, Smurf1 and Smurf2.

Report

(4 results)
  • 2013 Annual Research Report   Final Research Report ( PDF )
  • 2012 Research-status Report
  • 2011 Research-status Report
  • Research Products

    (40 results)

All 2013 2012 2011 2010 Other

All Journal Article (6 results) (of which Peer Reviewed: 3 results) Presentation (28 results) (of which Invited: 3 results) Book (6 results)

  • [Journal Article] Telmisartan ameliorates fibrocystic liver disease in an orthologous rat model of human autosomal recessive polycystic kidney disease.2013

    • Author(s)
      Yoshihara D, Kugita M, Sasaki M, Horie S, Nakanishi K, Abe T, Aukema HA, Yamaguchi T, Nagao S.
    • Journal Title

      PLoS One

      Volume: 8 Issue: 12 Pages: e81480-e81480

    • DOI

      10.1371/journal.pone.0081480

    • Related Report
      2013 Annual Research Report 2013 Final Research Report
    • Peer Reviewed
  • [Journal Article] 繊毛病(Ciliopathy)2012

    • Author(s)
      中西浩一,吉川徳茂
    • Journal Title

      日本小児腎臓病学会雑誌

      Volume: 25(2) Pages: 127-131

    • Related Report
      2013 Final Research Report
    • Peer Reviewed
  • [Journal Article] 常染色体劣性多発性嚢胞腎-疫学と臨床.特集:多発性嚢胞腎2012

    • Author(s)
      中西浩一,吉川徳茂,服部元史
    • Journal Title

      腎臓

      Volume: 34(3) Pages: 194-200

    • Related Report
      2013 Final Research Report
  • [Journal Article] ARPKDの基礎と臨床-最新の知見特集:多発性嚢胞腎-最新の知見と今後の課題2012

    • Author(s)
      中西浩一,吉川徳茂
    • Journal Title

      日本腎臓学会誌

      Volume: 54(4) Pages: 534-537

    • Related Report
      2013 Final Research Report
  • [Journal Article] 多発性嚢胞腎診療指針2011

    • Author(s)
      乳原善文, 香村衡一, 木村理, 嶋村剛, 田邉一成, 土谷健, 成田一衛, 中西浩一, 西尾妙織, 奴田原紀久雄, 野村信介, 花岡一成, 東原英二, 堀江重郎, 武藤智, 望月俊雄
    • Journal Title

      日本腎臓学会誌

      Volume: 53 Pages: 556-583

    • NAID

      10029736800

    • Related Report
      2011 Research-status Report
  • [Journal Article] 進行性腎障害に関する調査研究班多発性嚢胞腎分科会厚生労働省進行性腎障害調査研究班多発性嚢胞腎診療指針2010年8月(解説)2010

    • Author(s)
      乳原善文,香村衡一,木村理,嶋村剛,田邉一成,土谷健,成田一衛,中西浩一,西尾妙織,奴田原紀久雄,野村信介,花岡一成,東原英二,堀江重郎,武藤智,望月俊雄
    • Journal Title

      日本腎臓学会誌

      Volume: 53 Pages: 556-583

    • Related Report
      2013 Final Research Report
    • Peer Reviewed
  • [Presentation] 常染色体劣性多発性嚢胞腎の鑑別を要した3例2013

    • Author(s)
      浜武継,中西浩一,佐藤匡,向山弘展,戸川寛子,島友子,辻本弘,神波信次,吉川徳茂
    • Organizer
      第35回日本小児腎不全学会学術集会
    • Place of Presentation
      郡山
    • Year and Date
      2013-10-24
    • Related Report
      2013 Annual Research Report 2013 Final Research Report
  • [Presentation] cpkマウスARPKDモデルにおける病的Smad3リン酸化2013

    • Author(s)
      浜武継,中西浩一,向山弘展,戸川寛子,佐藤匡,島友子,宮嶋正康,野津寛大,高橋久英,長尾枝澄香,飯島一誠,吉川徳茂
    • Organizer
      第21回嚢胞性腎疾患研究会
    • Place of Presentation
      東京
    • Year and Date
      2013-09-21
    • Related Report
      2013 Final Research Report
  • [Presentation] cpkマウスにおける病的態・遺伝子診断小児嚢胞性腎疾患の病Smad3リン酸化2013

    • Author(s)
      浜武継,中西浩一,向山弘展,戸川寛子,佐藤匡,島友子,宮嶋正康,野津寛大,高橋久英,長尾枝澄香,飯島一誠,吉川徳茂
    • Organizer
      第22回発達腎研究会
    • Place of Presentation
      高槻
    • Year and Date
      2013-09-14
    • Related Report
      2013 Final Research Report
  • [Presentation] 遺伝性腎疾患のABC2013

    • Author(s)
      中西浩一
    • Organizer
      第14回徳島県小児膠原病・腎疾患談話会
    • Place of Presentation
      徳島(特別講演)
    • Year and Date
      2013-04-10
    • Related Report
      2013 Final Research Report
  • [Presentation] Smad3 Phosphorylated at Both Linker and COOH-Terminal Regions in Cyst-Lining Epithelia in cpk Mouse, a Model of ARPKD2013

    • Author(s)
      Hama T, Nakanishi K, Mukaiyama H, Togawa H, Sato M, Shima Y, Miyajima M, Nozu K, Nagao S, Takahashi H, Iijima K, Yoshikawa N
    • Organizer
      46^<th> Annual Meeting of the American Society of Nephrology
    • Place of Presentation
      Atlanta, USA(JASN 24:303A)
    • Related Report
      2013 Final Research Report
  • [Presentation] Possible contribution of fibrocytes to renal fibrosis in cpk mouse, a model of ARPKD2013

    • Author(s)
      Hama T, Nakanishi K, Mukaiyama H, Sato M, Togawa H, Shima Y, Miyajima M, Takahashi H, Nagao S, Iijima K, Yoshikawa N
    • Organizer
      15th Congress of the International Pediatric Nephrology Association
    • Place of Presentation
      Shanghai, China
    • Related Report
      2013 Final Research Report
  • [Presentation] cpkマウス多発性嚢胞腎モデル腎線維化におけるfibrocyteの関与2013

    • Author(s)
      浜武継,中西浩一,向山弘展,佐藤匡,戸川寛子,島友子,宮嶋正康,高橋久英,長尾静子,飯島一誠,吉川徳茂
    • Organizer
      第48回日本小児腎臓病学会学術集会
    • Place of Presentation
      徳島
    • Related Report
      2013 Annual Research Report 2013 Final Research Report
  • [Presentation] Ciliopathy. Continuous Professional Development, Theme: Current Issues in Pediatric Nephrology.2013

    • Author(s)
      Nakanishi K
    • Organizer
      The 11th Korea-Japan Pediatric Nephrology Seminar 2013
    • Place of Presentation
      Seoul, Korea
    • Related Report
      2013 Annual Research Report
    • Invited
  • [Presentation] Possible contribution of fibrocytes to renal fibrosis in cpk mouse, a model of ARPKD.2013

    • Author(s)
      Hama T, Nakanishi K, Mukaiyama H, Sato M, Togawa H, Shima Y, Miyajima M, Takahashi H, Nagao S, Iijima K, Yoshikawa N&#61486;
    • Organizer
      The 15th Congress of the International Pediatric Nephrology Association
    • Place of Presentation
      Shanghai, China
    • Related Report
      2013 Annual Research Report
  • [Presentation] Smad3 Phosphorylated at Both Linker and COOH-Terminal Regions in Cyst-Lining Epithelia in cpk Mouse, a Model of ARPKD.2013

    • Author(s)
      Hama T, Nakanishi K, Mukaiyama H, Togawa H, Sato M, Shima Y, Miyajima M, Nozu K, Nagao S, Takahashi H, Iijima K, Yoshikawa N.
    • Organizer
      The 46th Annual Meeting of the American Society of Nephrology
    • Place of Presentation
      Atlanta, USA
    • Related Report
      2013 Annual Research Report
  • [Presentation] アルポート症候群と多発性嚢胞腎2012

    • Author(s)
      中西浩一
    • Organizer
      第11回弘前小児腎疾患フォーラム
    • Place of Presentation
      弘前(特別講演)
    • Year and Date
      2012-10-26
    • Related Report
      2013 Final Research Report
  • [Presentation] 遺伝性ネフローゼ症候群と多発性嚢胞腎2012

    • Author(s)
      中西浩一
    • Organizer
      第22回北海道小児腎臓病研究会
    • Place of Presentation
      札幌(特別講演)
    • Year and Date
      2012-10-20
    • Related Report
      2013 Final Research Report
  • [Presentation] Possible Contribution of Fibrocytes to Renal Fibrosis in Cpk Mouse, a Model of ARPKD2012

    • Author(s)
      Hama T, Nakanishi K, Mukaiyama H, Togawa H, Shima Y, Miyajima M, Takahashi H, Nagao S, Iijima K, Yoshikawa N
    • Organizer
      45^<th> Annual Meeting of the American Society of Nephrology
    • Place of Presentation
      San Diego, USA(JASN 2012;23:596A)
    • Related Report
      2013 Final Research Report
  • [Presentation] ARPKDの病因・病態の解明と診断・治療の進歩2012

    • Author(s)
      中西浩一
    • Organizer
      第34回日本小児腎不全学会学術集会
    • Place of Presentation
      洲本(シンポジウム,座長兼任)
    • Related Report
      2013 Final Research Report
  • [Presentation] 繊毛病プロフェッショナル-52012

    • Author(s)
      中西浩一
    • Organizer
      第47回日本小児腎臓病学会学術集会
    • Place of Presentation
      東京(教育講演)
    • Related Report
      2013 Final Research Report
  • [Presentation] cpkマウスにおける部位特定的リン酸化Smad3の発現2012

    • Author(s)
      向山弘展,中西浩一,浜武継,戸川寛子,島友子,宮嶋正康,高橋久英,長尾枝澄香,飯島一誠,吉川徳茂
    • Organizer
      第47回日本小児腎臓病学会学術集会
    • Place of Presentation
      東京
    • Related Report
      2013 Final Research Report 2012 Research-status Report
  • [Presentation] 繊毛病2012

    • Author(s)
      中西浩一
    • Organizer
      第47回日本小児腎臓病学会学術集会
    • Place of Presentation
      東京
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] ARPKDの病態・遺伝子診断2012

    • Author(s)
      中西浩一
    • Organizer
      第34回日本小児腎不全学会学術集会
    • Place of Presentation
      洲本(淡路島)
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] Possible Contribution of Fibrocytes to Renal Fibrosis in Cpk Mouse, a Model of ARPKD2012

    • Author(s)
      Taketsugu Hama, Koichi Nakanishi, Hironobu Mukaiyama, Hiroko Togawa, Yuko Shima, Masayasu Miyajima, Hisahide Takahashi, Shizuko Nagao, Kazumoto Iijima, Norishige Yoshikawa
    • Organizer
      45th Annual Meeting of the American Society of Nephrology
    • Place of Presentation
      San Diego, USA
    • Related Report
      2012 Research-status Report
  • [Presentation] 小児における多発性嚢胞腎PKDの会2011

    • Author(s)
      中西浩一
    • Organizer
      第11回関西座談会
    • Place of Presentation
      大阪(講演)
    • Year and Date
      2011-11-20
    • Related Report
      2013 Final Research Report
  • [Presentation] cpkマウスにおける部位特定的リン酸化Smad3の発現2011

    • Author(s)
      浜武継,中西浩一,向山弘展,戸川寛子,島友子,宮嶋正康,高橋久英,長尾枝澄香,飯島一誠,吉川徳茂
    • Organizer
      第19回嚢胞性腎疾患研究会
    • Place of Presentation
      東京
    • Year and Date
      2011-09-11
    • Related Report
      2013 Final Research Report 2011 Research-status Report
  • [Presentation] Acceleration of Smad3 phosphorylation at linker regions via c-Jun NH2-terminal kinase (JNK) in cyst-lining epithelial cells in cpk mouse, a model of ARPKD2011

    • Author(s)
      Mukaiyama H, Nakanishi K, Hama T, Togawa H, Shima Y, Miyajima M, Takahashi H, Nagao S, Iijima K, Yoshikawa N
    • Organizer
      44^<th> Annual Meeting of the American Society of Nephrology
    • Place of Presentation
      Philadelphia, USA(JASN 22:576A)
    • Related Report
      2013 Final Research Report
  • [Presentation] cpkマウスARPKDモデルにおける上皮間葉移行(EMT)2011

    • Author(s)
      向山弘展,中西浩一,戸川寛子,濵武継,島友子,宮嶋正康,吉原大輔,長尾枝澄香,高橋久英,飯島一誠,吉川徳茂
    • Organizer
      第54回日本腎臓学会学術総会
    • Place of Presentation
      横浜
    • Related Report
      2013 Final Research Report
  • [Presentation] cpkマウスARPKDモデルにおける上皮間葉移行(EMT)2011

    • Author(s)
      向山弘展,中西浩一,戸川寛子,濵武継,島友子,宮嶋正康,吉原大輔,長尾枝澄香,高橋久英,飯島一誠,吉川徳茂
    • Organizer
      第52回日本腎臓学会学術総会
    • Place of Presentation
      横浜
    • Related Report
      2011 Research-status Report
  • [Presentation] Pathophysiology of polycystic kidney disease2011

    • Author(s)
      Nakanishi K
    • Organizer
      The 11th Asian Congress of Pediatric Nephrology 2011(招待講演)
    • Place of Presentation
      Fukuoka
    • Related Report
      2011 Research-status Report
  • [Presentation] Acceleration of Smad3 phosphorylation at linker regions via c-Jun NH2-terminal kinase (JNK) in cyst-lining epithelial cells in cpk mouse, a model of ARPKD.2011

    • Author(s)
      Mukaiyama H, Nakanishi K, Hama T, Togawa H, Shima Y, Miyajima M, Takahashi H, Nagao S, Iijima K, Yoshikawa N
    • Organizer
      The 44th Annual Meeting of the American Society of Nephrology
    • Place of Presentation
      Philadelphia
    • Related Report
      2011 Research-status Report
  • [Presentation] cpkマウスにおける病的Smad3リン酸化

    • Author(s)
      浜武継, 中西浩一, 向山弘展, 戸川寛子, 佐藤匡, 島 友子, 宮嶋正康, 野津寛大, 高橋久英, 長尾枝澄香, 飯島一誠, 吉川徳茂
    • Organizer
      第22回発達腎研究会
    • Place of Presentation
      高槻
    • Related Report
      2013 Annual Research Report
  • [Presentation] cpkマウスARPKDモデルにおける病的Smad3リン酸化

    • Author(s)
      浜武継, 中西浩一, 向山弘展, 戸川寛子, 佐藤匡, 島 友子, 宮嶋正康, 野津寛大, 高橋久英, 長尾枝澄香, 飯島一誠, 吉川徳茂
    • Organizer
      第21回嚢胞性腎疾患研究会
    • Place of Presentation
      東京
    • Related Report
      2013 Annual Research Report
  • [Book] 遺伝性嚢胞性疾患.臨床腎臓内科学2013

    • Author(s)
      中西浩一,吉川徳茂(安田隆,平和伸仁,小山雄太編)
    • Publisher
      南山堂 東京
    • Related Report
      2013 Final Research Report
  • [Book] 多発性嚢胞腎 14章 腎・尿細管機能異常 嚢胞性疾患 多発性嚢胞腎 「先天代謝異常ハンドブック」2013

    • Author(s)
      中西浩一,吉川徳茂(総編集 遠藤文夫 専門編集 山口清次,大浦敏博,奥山虎之)
    • Publisher
      中山書店 東京
    • Related Report
      2013 Final Research Report
  • [Book] 遺伝性嚢胞性疾患. 臨床腎臓内科学2013

    • Author(s)
      中西浩一, 吉川徳茂
    • Publisher
      南山堂
    • Related Report
      2013 Annual Research Report
  • [Book] 多発性嚢胞腎(小児) 「腎疾患治療マニュアル2012-2013」2012

    • Author(s)
      中西浩一,吉川徳茂( 編集 「腎と透析」編集委員会)
    • Publisher
      東京医学社
    • Related Report
      2013 Final Research Report
  • [Book] 第II部ARPKD(常染色体劣性多発性嚢胞腎) 多発性嚢胞腎診療ガイドQ&A2011

    • Author(s)
      中西浩一(監修 松尾清一 編集 堀江重郎)
    • Publisher
      診断と治療社
    • Related Report
      2013 Final Research Report
  • [Book] ARPKD(常染色体劣性多発性嚢胞腎) 多発性嚢胞腎診療ガイドQ&A2011

    • Author(s)
      中西浩一 監修 松尾清一 編集 堀江重郎
    • Publisher
      診断と治療社
    • Related Report
      2011 Research-status Report

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Published: 2011-08-05   Modified: 2019-07-29  

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