Figure 4.

Complementation of P2 fibroblasts with wild-type SDHA partially restores CII activity. Patient P2 and control (C) fibroblasts were transduced with lentiviruses carrying either the wild-type SDHA gene or GFP (green fluorescent protein) as a control. The activity of the different respiratory chain complexes and CV were analyzed in duplicate from two separate cell pellets of the stably selected cells. (a) Absolute measures of the different OXPHOS enzymes. Error bars represent SD values. (b) Efficiency of the complementation expressed as percentage of the GFP complemented controls. *P=0.02 in an unpaired t-test.