Efficacy of four selected aryl phosphoramidate Ppan derivatives in elevating hepatic CoA in the Pank1−/− mouse model. Hepatic CoA levels (pmol/mg liver) were determined for Pank1+/+ (WT) (n=14) and Pank1−/− (KO) (n=12) mice treated with vehicle alone (dark bars). Three control conditions consisted of Pank1−/− mice treated with 1.2 µmoles/g body weight of Pan (263 mg/kg; n=5), Ppan (359 mg/kg; n=6) or pantetheine (PanSH) (334 mg/kg; n=5). The four compounds that were positive in the fibroblast screen (Fig. 4) were tested at a dose of 1.2 µmoles/g body weight (Compound 1, 603 mg/kg; Compound 2, 569 mg/kg; Compound 4, 677 mg/kg; Compound 6, 752 mg/kg; n=9 per group, except for Compound 6 where n=2 due to limited quantities of material). Statistically significant differences with respect to KO values are presented as: * p<0.05; ** p<0.005; *** p<0.0005.