| 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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| 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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