Fig. 2. PCBP1^Δhep mice livers exhibit mitochondrial dysfunction.

A. Elongated and hyperfused mitochondria in hepatocytes from PCBP1^Δhep livers. Hepatocytes from PCBP1 fl/fl and Δhep mice were isolated and stained with mitochondrial-specific fluorescent dye Mitotracker green and subjected to live-cell confocal imaging. Inset (red) magnified in lower panels. B. Depletion of mitochondrial fusion protein mitofusin 2 in PCBP1^Δhep livers. Mfn2 levels measured by quantitative Western blot with VDAC as mitochondrial loading control. C. Depletion of mitochondrial respiratory complexes C II and C IV in PCBP1^Δhep livers. Western blot analysis of liver lysates probed with mixed antibodies for respiratory complexes I (NDUFB8), II (SDHB), III (UQCRC2), IV (MTCO1), and V (ATP5A). Quantification of C II and IV shown on right. D. ATP depletion in livers from PCBP1^Δhep mice. Data represent mean ± SD. * indicates p < 0.05, **p < 0.01, ***, p < 0.001 as determined by unpaired t-test (B, D) or 2-way ANOVA with Bonferroni multiple comparison test (C). E. Reduced mitochondrial oxygen consumption in hepatocytes from PCBP1^Δhep livers. Primary hepatocytes were isolated and mitostress test was performed to evaluate the mitochondrial oxygen consumption. Data from representative experiment are shown, see also Supplemental Fig. 2.