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

Figure 3

(a) Schematic representation of the three main COQ6 isoforms. Isoform a is the most widely represented and comprises exon 1a. Isoform b is transcribed from an alternative first exon (exon 1b) which is not translated, and translation starts in exon 2 using a different reading frame compared to those of isoforms a and c. Exon 3 is skipped and this allows restoration of the normal reading frame from the 3 exons. Isoform c is transcribed and translated from exon 1b. (b) Complex activity in wild-type (WT) and COQ6∆25 cells transduced with the empty vector (EV), COQ6 isoform a (Iso a), and COQ6 isoform c. . (c) HeLa cells stably expressing mtRFP were transiently transfected with COQ6-Iso-c-GFP plasmid expressing GFP fused to the C-terminus of COQ6.

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