Vanillic Acid Restores Coenzyme Q Biosynthesis and ATP Production in Human Cells Lacking COQ6

Figure 1

(a) PCR amplification of the COQ6 genomic region targeted by the CRISPR/Cas9 nucleases showing the 75 bp deletion in CRISPR/Cas9-treated cells. (b) Alignment of COQ6 proteins from different species. Boxed are the 25 amino acids affected by the deletion. The numbers refer to the human protein. (c) Three-dimensional structure of human COQ6. In red are the deleted amino acids, and in yellow are the bound FAD molecules. (d) Western blot analysis in wild-type and COQ6∆25 cells. (e) Steady-state CoQ₁₀ levels in wild-type and COQ6∆25 cells. (f) ¹⁴C 4-HB incorporation in wild-type and COQ6∆25 cells. The peaks corresponding to oxidized and reduced CoQ₁₀ are virtually undetectable in COQ6∆25 cells. An additional peak (red arrow) is present in COQ6∆25 cells. (g) Respiratory chain enzyme activities normalized to citrate synthase activity of COQ6∆25 cells. Data are expressed as percentage of activity of control cells.

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