CoQ Measurements in <i>Pdss2</i> Missense, Conditional Knockout, and Strain-Matched Control Mice.

<p>CoQ measurements in <i>Pdss2<sup>kd/kd</sup></i>; <i>Podocin/cre,Pdss2<sup>loxPloxP</sup></i>; <i>PEPCK/cre,Pdss2<sup>loxPloxP</sup></i>; <i>Alb/cre,Pdss2<sup>loxP/loxP</sup></i>; and strain-matched control mice (B6 and B6.<i>Pdss2<sup>loxPloxP</sup></i>). <i>A</i>, Kidney levels of CoQ<sub>9</sub> and CoQ<sub>10</sub> are significantly lower in B6.<i>Pdss2<sup>kd/kd</sup></i> (missense) mice than in B6 control mice. <i>B</i>, Kidney levels of CoQ<sub>9</sub> and CoQ<sub>10</sub> are similar in B6.<i>Podocin/cre,Pdss2<sup>loxPloxP</sup></i>; <i>B6.PEPCK/cre,Pdss2<sup>loxPloxP</sup></i>; B6.<i>Alb/cre,Pdss2<sup>loxP/loxP</sup></i>; and control mice. The 92-day-old controls are B6.<i>Pdss2<sup>loxP/+</sup></i>, and the 94-day-old control is B6.<i>Pdss2<sup>loxP/loxP</sup></i>. <i>C</i>, Liver levels of CoQ<sub>9</sub> in B6.<i>Alb/cre,Pdss2<sup>loxP/loxP</sup></i> mice were below 30 picomoles/mg protein (*) while these same levels in B6.<i>Podocin/cre,Pdss2<sup>loxPloxP</sup></i> and B6.<i>PEPCK/cre,Pdss2<sup>loxPloxP</sup></i> mice were similar to those in control mice. The strains of the control mice in are the same as those in panel <i>B</i>.</p>