| Project/Area Number |
25293211
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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| Allocation Type | Partial Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Metabolomics
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| Research Institution | National Cardiovascular Center Research Institute (2015-2016)
Kyoto University (2013-2014) |
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Principal Investigator |
Hosoda Kiminori 国立研究開発法人国立循環器病研究センター, 病院, 部長 (40271598)
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| Co-Investigator(Renkei-kenkyūsha) |
TAKAHASHI Kazutoshi 京都大学, iPS細胞研究所, 講師 (80432326)
YAMASHITA Jun 京都大学, iPS細胞研究所, 教授 (50335288)
NAKAO Kazuki 東京大学, 医学研究科, 准教授 (20217657)
Ebihara Ken 自治医科大学, 医学研究科, 准教授 (70362514)
SONE Masakatssu 京都大学, 医学研究科, 特定准教授 (40437207)
SAKURAI Hidetoshi 京都大学, iPS細胞研究所, 講師 (80528745)
FUJIKURA Junji 京都大学, 医学研究科, 助教 (70378743)
NOGUCHI Michio 京都大学, 医学研究科, 特定講師 (00432394)
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| Project Period (FY) |
2013-04-01 – 2017-03-31
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| Project Status |
Completed (Fiscal Year 2016)
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| Budget Amount *help |
¥18,460,000 (Direct Cost: ¥14,200,000、Indirect Cost: ¥4,260,000)
Fiscal Year 2015: ¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000)
Fiscal Year 2014: ¥6,240,000 (Direct Cost: ¥4,800,000、Indirect Cost: ¥1,440,000)
Fiscal Year 2013: ¥6,890,000 (Direct Cost: ¥5,300,000、Indirect Cost: ¥1,590,000)
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| Keywords | seipin / iPS細胞 / 脂肪細胞 / 脂肪萎縮症 / インスリン抵抗性 / レスキュー / 脂肪細胞分化 / 先天性脂肪萎縮症 / 成因解明 |
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| Outline of Final Research Achievements |
Congenital generalized lipodystrophy (CGL) is an autosomal recessive disorder characterized by marked scarcity of adipose tissue, extreme insulin resistance, hypertriglyceridemia, hepatic steatosis and early-onset diabetes. Mutation of the BSCL2/SEIPIN gene causes congenital generalized lipodystrophy. iPS cells were generated from two patients with CGL. Upon adipogenic differentiation, BSCL2-iPS cells exhibited marked reduction of lipid droplet formation concomitant with diffuse cytoplasmic distribution of ADRP, compared with iPS cells from healthy individuals. Forced expression of BSCL2 not only rescued the lipid accumulation defects, but also restored cytoplasmic punctate localization of ADRP in BSCL2-iPS cells. Coimmunoprecipitation indicated SEIPIN interacted with ADRP.
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