Investigation of abnormal lipid metabolism in Niemann-Pick disease type C based on multiple methodologies
| Project/Area Number |
18K15699
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| Research Category |
Grant-in-Aid for Early-Career Scientists
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| Allocation Type | Multi-year Fund |
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| Review Section |
Basic Section 52050:Embryonic medicine and pediatrics-related
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| Research Institution | Tohoku University |
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Principal Investigator |
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| Project Period (FY) |
2018-04-01 – 2020-03-31
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| Project Status |
Completed (Fiscal Year 2019)
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| Budget Amount *help |
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2019: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2018: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
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| Keywords | ニーマンピック病 / 脂質解析 / LC/MS/MS / 質量分析 / コレステロール / スフィンゴ脂質 / 新規リン脂質 / 化学診断 / ニーマンピック病C型 / 構造解析 / 脂質 / コレステロール代謝物 / 新規クラスリン脂質 / LC-MS/MS / 脂質異常 / ライソゾーム病 / スフィンゴ脂質代謝物 / LC/ESI-MS/MS / リピドミクス |
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| Outline of Final Research Achievements |
In this study, we analyzed the characteristic lipid alterations to Niemann-Pick type C (NPC), the localization of intracellular abnormal metabolism, and the comprehensive variation analysis of lipid metabolism.As a result of structural analysis of Lysosphingomyelin-509 using derivatization method and mass spectrometry method, it was identified as a novel lipid N-palmitoyl-O-phosphocholine-serine.Next, we examined various fluorescent staining reagents for intracellular organelles and confirmed that the intracellular organelle fraction could be traced correctly.For various lipids, we found a characteristic lipid variation in NPC patients using metabolomics analysis methods.
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| Academic Significance and Societal Importance of the Research Achievements |
本研究の成果で、未知脂質lysosphingomyelin-509の構造を決定する過程で、当該分子をN-palmitoyl-O-phosphocholine-serine (PPCS) と明らかにした。PPCSはデータベースに無い新規分子であり、こうした新たな分子を発見できたことは生化学的にも意義が大きい。さらに、網羅的解析からも新たな分子変化が見出されており、今後さらなる解析が期待される。PPCSはニーマンピック病の診断に極めて有用であり、今後の本疾患の診断に役立てられるものと考えられる。
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Report
(3 results)
Research Products
(27 results)
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[Journal Article] Structural Determination of Lysosphingomyelin-509 and Discovery of Novel Class Lipids from Patients with Niemann-Pick Disease Type C2019
Author(s)
Masamitsu Maekawa, Isamu Jinnoh, Yotaro Matsumoto, Aya Narita, Ryuichi Mashima, Hidenori Takahashi, Anna Iwahori, Daisuke Saigusa, Kumiko Fujii, Ai Abe, Katsumi Higaki, Shosei Yamauchi, Yuji Ozeki, Peter T Clayton, Hiroaki Yamaguchi, Nariyasu Mano, et al.
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Journal Title
International Journal of Molecular Sciences
Volume: 20
Issue: 20
Pages: 5018-5018
DOI
Related Report
Peer Reviewed / Open Access / Int'l Joint Research
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[Journal Article] Investigation of diagnostic performance of five urinary cholesterol metabolites for Niemann-Pick disease type C2019
Author(s)
Masamitsu Maekawa, Isamu Jinnoh, Aya Narita, Takashi Iida, Daisuke Saigusa, Anna Iwahori, Hiroshi Nittono, Torayuki Okuyama, Yoshikatsu Eto, Kousaku Ohno, Peter T. Clayton, Hiroaki Yamaguchi, Nariyasu Mano
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Journal Title
Journal of Lipid Research
Volume: 60
Issue: 12
Pages: 2074-2081
DOI
Related Report
Peer Reviewed / Int'l Joint Research
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