2013 Fiscal Year Final Research Report
Elucidation of the synthetic mechanism, disorders, and physiological functions of very long-chain fatty acids
| Project/Area Number |
23370057
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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 |
Functional biochemistry
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| Research Institution | Hokkaido University |
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Principal Investigator |
KIHARA Akio 北海道大学, 薬学研究科(研究院), 教授 (50333620)
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| Co-Investigator(Kenkyū-buntansha) |
SASSA Takayuki 北海道大学, 大学院・薬学研究院, 講師 (20342793)
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| Co-Investigator(Renkei-kenkyūsha) |
OBARA Keisuke 北海道大学, 大学院・薬学研究院, 助教 (30419858)
OHNO Yusuke 北海道大学, 大学院・薬学研究院, 助教 (50611498)
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| Project Period (FY) |
2011-04-01 – 2014-03-31
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| Keywords | 脂質 / 代謝 / 脂肪酸 / 生体膜 / 極長鎖脂肪酸 |
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| Research Abstract |
Although cellular level of very long-chain fatty acids (VLCFAs), which have carbon chain-length greater than 20 (>C20), is less abundant than that of long-chain fatty acids (LCFAs; C11-C20), VLCFAs possess important functions that cannot be substituted for by LCFAs and related to some disorders. In this research project, we revealed the functions of VLCFAs in skin barrier formation, relationship between metabolism of VLCFAs and several disorders such as adrenoleucodystrophy, myopathy, and mental retardation, regulatory mechanism of the VLCFA synthetic enzymes by the ceramide synthases, and the roles of VLCFAs in the vesicular transport via endosomes, by physiological and cell biological analyses using knockout mice and cultured cells, by biochemical assays using purified VLCFA synthetic enzymes, and by yeast genetical approach.
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[Journal Article] Impaired epidermal permeability barrier in mice lacking the Elovl1 gene responsible for very long-chain fatty acid production2013
Author(s)
Sassa T, Ohno Y, Suzuki S, Nomura T, Nishioka C, Kashiwagi T, Hirayama T, Akiyama M, Taguchi R, Shimizu H, Itohara S, Kihara A
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Journal Title
Mol. Cell. Biol
Volume: 33
Pages: 2787-2796
DOI
Peer Reviewed
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[Journal Article] Congenital myopathy is caused by mutation of HACD12013
Author(s)
Muhammad E, Reish O, Ohno Y, Scheetz T, Deluca A, Searby C, Regev M, Benyamini L, Fellig Y, Kihara A, Sheffield VC, Parvari R
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Journal Title
Hum. Mol. Genet.
Volume: 22
Pages: 5229-5236
DOI
Peer Reviewed
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