Figure 3.

Mitochondrial energetic function is altered and CoQ level is decreased in rmd. (A) Mitochondrial respiratory chain enzyme activities in rmd were compared with healthy littermates. C1, Complex I ; C2, Complex II ; C3, Complex III ; C4, Complex IV (n = 4). (B) The rate of ATP synthesis measured by luminometry method (n = 4). (C) Total CoQ9 level (littermate forelimb, n = 13; littermate hindlimb, n = 12; rmd forelimb, n = 11; rmd hindlimb, n = 13). Data are expressed as the mean ± SD of experiment number shown as n. *P < 0.05, **P < 0.005, ***P < 0.001.