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. Author manuscript; available in PMC: 2016 Dec 1.
Published in final edited form as: Mol Genet Metab. 2015 Oct 29;116(4):281–288. doi: 10.1016/j.ymgme.2015.10.011

Fig. 5.

Fig. 5

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.