Figure 4. Respirometry and H₂O₂ production in mitochondria with RISP depleted CIII (A-C), and signs of oxidative stress in liver tissue (D, E).

(A) Blue Native PAGE and Western blot showing decreased Rieske iron sulphur protein (RISP) incorporation in CIII in mitochondria from two representative Bcs1l ^G/G (G/G₁, G/G₂) mice compared to wild type (A/A₁, A/A₂) aged >30 d. (B) Respiratory chain oxygen consumption under convergent substrate input in CI and CII is impaired in G/G₁. (C) Similar H₂O₂ production was detected with Amplex Red in isolated mitochondria of G/G₁ and A/A₁ mice. (D) In liver tissue homogenates of three age group animals, metabolomic analyses showed that log₂ fold changes of the oxidative stress markers were significantly increased in sick animals only (>30 days). (E) Antioxidant defence in liver tissue measured with quantitative PCR in 14 days old and sick (>30 d) homozygotes expressed as relation to β-actin. In sick animals manganese superoxide dismutase (MnSOD) and catalase (Cat) were decreased, whereas glutathione peroxidase 3 (GPx-3) was increased.