Figure 6.

Evaluation of Mitochondria

(A) Graphs showing activity levels of respiratory chain complexes in neurons at 35 DIV measured through spectrophotometric analysis. Values are normalized for the activity of citrate synthase.

(B) Western blot performed on all neuronal lines at 35 DIV assessing the amount of respiratory chain complexes, TOMM20, and actin.

(C) Graphs showing the amount of the complexes of respiratory chain in neurons at 35 DIV measured through western blot and normalized for actin.

(D) Graphs showing the amount of the structural mitochondrial protein TOMM20 in neurons at 35 DIV measured through western blot and normalized for actin.

(E) Graphs showing mtDNA content in neurons at 35 DIV, measured by qPCR through the simultaneous detection of the mitochondrial gene CytB and the nuclear reference gene APP.

(F) Graphs showing CoQ10 levels in neurons at 35 DIV, measured through HPLC.

(G) Graphs showing the amount of nine enzymes involved in the synthesis of CoQ10 in neurons at 35 DIV, measured through western blot and normalized for vinculin: PDSS1, PDSS2, COQ2, COQ4, COQ5, COQ6, COQ7, ADCK3/COQ8A, and COQ9. AT, affected twin; UT, unaffected twin.

Data are expressed as mean ± SEM. ^∗p < 0.05; ^∗∗p < 0.01; ^∗∗∗p < 0.001. Three independent experiments were performed for each clone and graphs represent pooled data from all the experiments. Two clones were used for each subject, with the exception of a few cases (single clone). Raw data showing results for each single clone are reported in Figures S6 and S7. See also Figures S2 and S4.