Figure 5. Increased H₂O₂ Emissions and Decreased Mitochondrial Respiration in Skeletal Muscle From Sibling 2.

(A) High-resolution respiratory flux per mg of saponin-permeabilized vastus lateralis muscle from COQ7-affected sibling 2 and unrelated controls. FCCP-induced maximal respiration and CIV activity were lower in the COQ7-affected muscle. (B) Simultaneous fluorometric quantification of H₂O₂ emissions was higher in vastus lateralis from affected sibling 2. (C) BN-PAGE analysis revealed a decrease in CI and CIV mitochondrial supercomplexes in vastus lateralis muscle of affected sibling 2. BN‐PAGE = blue native PAGE.