Hexacosenoyl-CoA is the most abundant very long-chain acyl-CoA in ATP binding cassette transporter D1-deficient cells

• DOI: 10.1194/jlr.p119000325
• Publication Date: 2020-02-19T20:44:30Z
• Authors: Kotaro Hama; Yuko Fujiwara; Shigeo Takashima; Yasuhiro Hayashi; Atsushi Yamashita; Nobuyuki Shimozawa; Kazuaki Yokoyama
• Keywords: Acyl-CoA

X-linked adrenoleukodystrophy (X-ALD) is an inherited disorder caused by deleterious mutations in the ABCD1 gene. The protein transports very long-chain FAs (VLCFAs) from cytosol into peroxisome where VLCFAs are degraded through β-oxidation. dysfunction leads to VLCFA accumulation individuals with X-ALD. activated esterification CoA before metabolic utilization. However, intracellular pools and profiles of individual acyl-CoA esters have not been fully analyzed. In this study, we profiled species fibroblasts X-ALD patients ABCD1-deficient HeLa cells. We found that hexacosenoyl (26:1)-CoA, but hexacosanoyl (26:0)-CoA, was most abundantly concentrated among VLCFA-CoA these also show 26:1-CoA mainly synthesized oleoyl-CoA, turnover rate almost identical oleoyl-CoA both WT findings our study provide precise quantitative information each living Our results suggest endogenously as a FA elongation pathway then efficiently converted other metabolites, such phospholipids, absence ABCD1.