| Project/Area Number |
19390045
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Medical pharmacy
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| Research Institution | Kumamoto University |
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Principal Investigator |
KAI Hirofumi Kumamoto University, 大学院・生命科学研究部, 教授 (30194658)
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| Co-Investigator(Kenkyū-buntansha) |
首藤 剛 熊本大学, 大学院・医学薬学研究部, 助手 (80333524)
スイコ メリー・アン・ソテン 熊本大学, 大学院・医学薬学研究部, 助教 (20363525)
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| Co-Investigator(Renkei-kenkyūsha) |
SHUTO Ysuyoshi 熊本大学, 大学院・生命科学研究部, 講師 (80333524)
SUICO MARY Ann Soten 熊本大学, 大学院・生命科学研究部, 助教 (20363525)
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| Project Period (FY) |
2007 – 2009
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| Project Status |
Completed (Fiscal Year 2009)
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| Budget Amount *help |
¥18,980,000 (Direct Cost: ¥14,600,000、Indirect Cost: ¥4,380,000)
Fiscal Year 2009: ¥5,720,000 (Direct Cost: ¥4,400,000、Indirect Cost: ¥1,320,000)
Fiscal Year 2008: ¥5,720,000 (Direct Cost: ¥4,400,000、Indirect Cost: ¥1,320,000)
Fiscal Year 2007: ¥7,540,000 (Direct Cost: ¥5,800,000、Indirect Cost: ¥1,740,000)
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| Keywords | 薬理学 / タンパク質 / 遺伝病 / cystic fibrosis / FAP / CFTR / transthyretin / phosphatidic acid / mucin / 小胞体 / アミロイド / ENaC / 嚢胞性線維症(CF) / 家族性アミロイドポリニューロパチー(FAP) / calnexin(CNX) / transthyretin(TTR) / 小胞体関連分解(ERAD) / BiP / calnexin / toll-like receptor |
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| Research Abstract |
Drug development for cystic fibrosis and familial amyloid polyneuropathy is still in progress. We found out the intracellular protein quality control mechanisms of ΔF508 CFTR, a major mutant of cystic fibrosis, and of transthyretin variants, resulting in proposal of new concept of drug development for familial amyloid polyneuropathy. In addition, we found that the endoplasmic reticulum-associated degradation of transthyretin variants is negatively regulated by BiP in mammalian cells.
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