|Budget Amount *help
¥39,780,000 (Direct Cost: ¥30,600,000、Indirect Cost: ¥9,180,000)
Fiscal Year 2017: ¥8,060,000 (Direct Cost: ¥6,200,000、Indirect Cost: ¥1,860,000)
Fiscal Year 2016: ¥9,620,000 (Direct Cost: ¥7,400,000、Indirect Cost: ¥2,220,000)
Fiscal Year 2015: ¥15,470,000 (Direct Cost: ¥11,900,000、Indirect Cost: ¥3,570,000)
Fiscal Year 2014: ¥6,630,000 (Direct Cost: ¥5,100,000、Indirect Cost: ¥1,530,000)
|Outline of Final Research Achievements
In the fatty acid elongation cycle, which produces very long-chain fatty acids, four reactions are catalyzed by ELOVL1-7, KAR, HACD1-4, and TER. In the present study, we elucidated the molecular mechanism of myopathy and the differences in the substrate specificity of HACD1-4, using knockout mice or knockout cells of HACD genes. Analyses using ELOVL mutant mice and biochemical assays revealed the substrate specificity of ELOVLs and the mechanism of activity regulation of ELOVL6 by KAR. We identified fatty acid omega-hydroxylase CYP4F22 as the gene involved in the production of the very long-chain lipid acylceramide, which is important for skin barrier formation, and elucidated the molecular mechanism of ichthyosis. We revealed a new function of TER other than fatty acid elongation.