CoQ Measurements in Pdss2 Missense, Conditional Knockout, and Strain-Matched Control Mice.

CoQ measurements in Pdss2kd/kd; Podocin/cre,Pdss2loxPloxP; PEPCK/cre,Pdss2loxPloxP; Alb/cre,Pdss2loxP/loxP; and strain-matched control mice (B6 and B6.Pdss2loxPloxP). A, Kidney levels of CoQ9 and CoQ10 are significantly lower in B6.Pdss2kd/kd (missense) mice than in B6 control mice. B, Kidney levels of CoQ9 and CoQ10 are similar in B6.Podocin/cre,Pdss2loxPloxP; B6.PEPCK/cre,Pdss2loxPloxP; B6.Alb/cre,Pdss2loxP/loxP; and control mice. The 92-day-old controls are B6.Pdss2loxP/+, and the 94-day-old control is B6.Pdss2loxP/loxP. C, Liver levels of CoQ9 in B6.Alb/cre,Pdss2loxP/loxP mice were below 30 picomoles/mg protein (*) while these same levels in B6.Podocin/cre,Pdss2loxPloxP and B6.PEPCK/cre,Pdss2loxPloxP mice were similar to those in control mice. The strains of the control mice in are the same as those in panel B.