Diagnosis and molecular mechanisms for hypofibrinogenemia inducing liver cirrhosis
| Project/Area Number |
26460672
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Laboratory medicine
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| Research Institution | Shinshu University |
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Principal Investigator |
OKUMURA Nobuo 信州大学, 学術研究院保健学系, 教授 (60252110)
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| Co-Investigator(Kenkyū-buntansha) |
寺澤 文子 北陸大学, 新学部設置準備室, 教授 (40109210)
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| Research Collaborator |
ARAI Shinpei
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| Project Period (FY) |
2014-04-01 – 2017-03-31
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| Project Status |
Completed (Fiscal Year 2016)
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| Budget Amount *help |
¥4,940,000 (Direct Cost: ¥3,800,000、Indirect Cost: ¥1,140,000)
Fiscal Year 2016: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2015: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2014: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
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| Keywords | フィブリノゲン低下症 / フィブリノゲン蓄積症 / 細胞質封入体 / 粗面小胞体 / 蛍光抗体法 / プロテアソーム阻害剤 / オートファジー阻害剤 / アポトーシス促進剤 / オートファジー促進剤 / フィブリノゲン異常症 / 肝小胞体蓄積病 / 細胞内封入体 / CHO細胞 / HuH細胞 |
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| Outline of Final Research Achievements |
Six hypofibrinogenemic mutations have been identified in gamma-chain of fibrinogen for fibrinogen storage disease (FSD). To investigate the diagnostic test and pharmaceutical therapy for FSD, we performed research. First we established aberrant fibrinogen-producing Chinese hamster ovary (CHO) cells and observed those by immunofluorescence method using anti-fibrinogen antibody. All of six transiently transfected CHO cell lines specifically showed fibrous forms of intracellular inclusion bodies.
To analyze the mechanisms for the formation and inhibition of fibrous forms of inclusion bodies, some of anticipated inhibitor or enhancer reagents were added into the established cell lines. These results suggest that fibrous inclusion bodies are formed by the inhibition of proteasome and/or autophagy protein degradation pathway, however, enhancer reagents for these pathway does not reduce the fibrous forms. Therefore these enhancing reagents can not use as the therapeutic agent for FSD.
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Report
(4 results)
Research Products
(4 results)
