Figure 3.

High-resolution respirometry in fibroblasts reveals mild complex I defects. Results of high-resolution respirometry in fibroblasts from the patient (n = 5) compared to 20 control fibroblast samples. There are decreased parameters relating to complex I activity: when stimulated with ADP the rates with the substrates pyruvate and glutamate are decreased, as is the rotenone-inhibited component of the uncoupled rate, which is complex I specific. In contrast, the rate is excessively increased when a complex II substrate succinate is added as reflected in the succinate/glutamate ratio. * p < 0.05, ** p < 0.01 by Student t-testing.