Mpv17 ablation in CFW mice causes liver specific mtDNA depletion and deficiency of all respiratory chain complexes that contain mtDNA-encoded subunits.

<p><b>(A-C)</b> Mpv17 expression (<b>A</b>), mtDNA copy number (<b>B</b>) and steady state levels of OXPHOS subunits (<b>C</b>) in the liver, kidney and brain of wild-type (WT, <i>Mpv17</i><sup><i>+/+</i></sup><i>)</i> and knockout (KO, <i>Mpv17</i><sup><i>-/-</i></sup><i>)</i> mice. (<b>B</b>) Quantification of mtDNA in WT and KO mice. Data are expressed as mean ± SEM of n = 6. (Student test, *** P<0.001, NS, not significant, p>0.05). (<b>D</b>) BN-PAGE analysis of OXPHOS complexes in the liver of WT and KO mice. Sup-C, supercomplexes, Sub-C, subcomplexes of the OXPHOS system. Vdac levels were used as a loading control on a 12% SDS-PAGE gel.</p>