| Budget Amount *help |
¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2019: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2018: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2017: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2016: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2015: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
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| Outline of Final Research Achievements |
VLCFA are transported by ABCD1 into the peroxisome where they are degraded by β-oxidation. ABCD1 is the causative gene of X-ALD. Fatty acyl-coenzyme A (acyl-CoA) is an active form and serves as metabolic intermediates of fatty acids. Acyl-CoA is composed of both a hydrophobic fatty acyl moiety and a hydrophobic CoA joined with a thioester linkage. The intracellular pool of each acyl-CoA ester has not yet been fully analyzed. In this study, we profiled the acyl-CoA species in fibroblasts from X-ALD patients and in ABCD1-deficient HeLa cells. We found that hexacosenoyl (26:1)-CoA was the most abundantly concentrated among the VLCFA-CoA species in these cells. We also show that 26:1-CoA is mainly synthesized from oleoyl-CoA, and the metabolic turnover rate of 26:1-CoA was almost identical to that of oleoyl-CoA in both wild-type and ABCD1-deficient HeLa cells. The findings of our study provide quantitative and metabolic information of each acyl-CoA species in living cells.
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