Table 2. .

Biochemical Assays for COQ₁₀ Biosynthesis

Fibroblasts	CoQ10 Biosynthesisa	% of Control Mean
Assay 1b:
Control	3,324 ± 526 DPM/mg protein/d	100
Patient	402 ± 83 DPM/mg protein/d	12
Assay 2c:
Control	14.29 ± 2.3 pmol/mg protein/h	100
Patient	11.5 ± 0.5 pmol/mg protein/h	81

^aIn the three assays, radiolabeled COQ₁₀ was isolated by HPLC and was quantitated in a scintillation counter. Controls are measured as means ± SDs (n=5). Patient measurements were realized per duplicated and the results are shown as means ± SDs. DPM = decays per minute.

^bCultured cells were incubated for 48 h with 0.1 μCi ¹⁴C-PHB (50 Ci/mol specific activity).

^cFibroblast homogenates were incubated with ³H-decaprenyl-PP and PHB for 1 h.