Elucidation of the synthetic mechanism, disorders, and physiological functions of very long-chain fatty acids

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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.