2020 Fiscal Year Final Research Report
Molecular mechanism of novel phospholipid transport proteins and its relationship with mitochondrial diseases
| Project/Area Number |
17K08642
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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 |
General medical chemistry
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| Research Institution | Dokkyo Medical University |
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Principal Investigator |
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| Project Period (FY) |
2017-04-01 – 2021-03-31
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| Keywords | リン脂質 / ミトコンドリア / 筋分化 |
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| Outline of Final Research Achievements |
Mitochondrial membranes are composed of two phospholipid bilayers. More than half of the phospholipids in mitochondrial membranes are phosphatidylcholine (PC). Mitochondria lack the enzymes required for PC synthesis. The lipid is therefore likely supplied from organelles such as the endoplasmic reticulum which contains the biosynthesis systems for PC. This study clarified the molecular basis for transporting PC to mitochondria by StarD7. This study also found that deficiency of StarD7 in myoblast caused not only impairement of mitochondrial functions but also inhibition of myogenic differentiation.
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| Free Research Field |
脂質生化学
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| Academic Significance and Societal Importance of the Research Achievements |
ERなどのオルガネラからミトコンドリアへのPC輸送機構は細胞生物学や脂質生化学において重要な研究課題の一つであるが、長い間解明されていなかった。今回、研究代表者が先行研究で見出したタンパク質StarD7が上記輸送機構の一端にかかわることを分子のレベルで明らかにした。ところで、ミトコンドリア病を引き起こす原因遺伝子の変異は未だ多くが不明である。本研究ではStarD7によるPC輸送機構が破綻すると、ミトコンドリアの機能低下が生じ、筋細胞の分化能が著しく阻害されミオパチーの原因になり得ることを示唆した。
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