Increased Mitochondrial Protein Levels and Bioenergetics in the Musculus Rectus Femoris of Wfs1-Deficient Mice

Figure 1

(a) Original recording of the assessment of the respiratory chain function in skinned muscle fibers. Additions: F: fibers; ADP: 2 mM ADP; Rot: 10 μM rotenone; Succ: 10 mM succinate; Atr: 0.1 mM atractyloside; Ant: 10 μM antimycin A; TMPD + Asc: 0.5 mM TMPD with 2 mM ascorbate; NaN₃: 5 mM sodium azide. Upper, blue line: [O₂]; lower, red line: O₂ flux. Summary of results, which correspond to the experimental scheme shown in (a), is given in groups of permeabilized muscle fibers from the heart (b), musculus soleus (c), and musculus rectus femoris (d). V₀: basal respiration; V_ADP: complex I- and ADP-dependent respirations; V_Succ: complex II-dependent respiration in the presence of rotenone with succinate; V_Atr: atractyloside-insensitive respiration rate; V_TMPD: respiration with TMPD and ascorbate in the presence of antimycin A; V_NaN3: respiration in the presence of sodium azide; V_Leak: the difference between V_Atr and V_Ant, proton leak-dependent respiration; V_COX: sodium azide-sensitive portion of the respiration rate in the presence of TMPD and ascorbate. Data are mean ± SEM. Compared to wild type: , , and .

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