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

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posted on 2016-01-18, 15:33 authored by Ilaria Dalla Rosa, Yolanda Cámara, Romina Durigon, Chloe F. Moss, Sara Vidoni, Gokhan Akman, Lilian Hunt, Mark A. Johnson, Sarah Grocott, Liya Wang, David R. Thorburn, Michio Hirano, Joanna Poulton, Robert W. Taylor, Greg Elgar, Ramon Martí, Peter Voshol, Ian J. Holt, Antonella Spinazzola

(A-C) Mpv17 expression (A), mtDNA copy number (B) and steady state levels of OXPHOS subunits (C) in the liver, kidney and brain of wild-type (WT, Mpv17+/+) and knockout (KO, Mpv17-/-) mice. (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). (D) 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.

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