Fig. 1: Impact of LRPPRC deficiency on mitochondrial biogenesis, mitochondrial ribosome content, and proteostatic systems. | Communications Biology

Fig. 1: Impact of LRPPRC deficiency on mitochondrial biogenesis, mitochondrial ribosome content, and proteostatic systems.

From: Adaptive optimization of the OXPHOS assembly line partially compensates lrpprc-dependent mitochondrial translation defects in mice

Fig. 1

a mitochondrial DNA copy number measured in whole liver homogenates from of H-Lrpprc+/+ and H-Lrpprc−/− mice at 5 weeks of age. Values represent means ± sem of the ND1/HK2 ΔCt ratio (n = 4). Student T-test was used to assess significance (**P < 0.01). b Transmission Electron Micrographs of H-Lrpprc+/+ and H-Lrpprc−/− livers at 5 weeks of age (magnification 1900x). c PGC1α and TFAM protein expression in whole liver homogenates from H-Lrpprc+/+ and H-Lrpprc−/− mice. d, e Proteomic profiling of Large 39 S and Small 29 S mitochondrial ribosomal proteins (MRPL and MRPS proteins) in digitonin-solubilized mitochondria from H-Lrpprc+/+ and H-Lrpprc−/− mice following separation by hybrid CN/BN-PAGE. The heatmap (d) represents the average abundance (n = 3 per group) of individual ribosomal protein in each of the gel bands analyzed relative to the maximal abundance observed for this protein in all samples analyzed. Each lane represents an individual subunit, and the range of molecular weight covered by each band is indicated at the bottom. To represent the abundance of mitoribosomes, iBAQ intensities for all of the large and small ribosomal subunits detected in each band were summed for each experimental replicate and the average values obtained for each genotype is presented in stacked histograms (e). f Total abundance of selected mitochondrial proteases in digitonin-solubilized mitochondria from H-Lrpprc+/+ and H-Lrpprc−/− mice. For a given protein, total abundance in a sample was calculated by summing the normalized iBAQ intensities observed in each band analyzed. Values presented represent the mean ± sem for n = 3 in each group. Validation immunoblots for selected proteases are shown in Fig. S1. One sample T test corrected for multiple comparisons were performed to assess significance (***Q < 0.01, **Q < 0.01, * Q < 0.05). g transcript level of Chop normalized to Tbp as a housekeeping gene in whole liver homogenates from H-Lrpprc+/+ (n = 4) and H-Lrpprc−/− (n = 7).

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