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. 2016 Nov 17;9(1):96–111. doi: 10.15252/emmm.201606356

Figure 2. Schematic representation of CoQ biosynthesis showing the molecular defects in patient fibroblasts (P1–P5).

Figure 2

The isoprene tail is synthesized from farnesyl pyrophosphate by PDSS1/PDSS2 and condensed to the 4HB ring by COQ2. The ring is processed into CoQ by COQ3‐7. ADCK3/CABC1 (COQ8) regulates CoQ biosynthesis through phosphorylation of COQ3, COQ5, and COQ7.