Deficits in the respiratory chain and the tricarboxyl acid cycle were present in P301S transgenic mice. Mitochondrial enzyme activity of CS, CI, SDH, GR, and ACO, and protein expression of cytochrome c, MnSOD, total SOD (SOD), CI, CIII, CIV (COXIV) and ATPase at 7 mo (A, B) and 10 mo (C, D) in male (A, C) and female (B, D) P301S (Tg) mice and their wild-type (Wt) littermates. Data are normalized per milligram of protein and expressed as means ± se. Inset: mitochondrial enzyme activity of CI, SDH, and GR after normalization by CS at 7 mo of age between male P301S mice and their wild-type male littermates (A). Starting at 7 mo, P301S mice had mitochondrial abnormalities compared to their wild-type littermates.