Figure 2.

Enzymology in control and PMD patient fibroblast cell lines. Control and PMD patient fibroblasts were measured CS and respiratory chain complex I–IV (CI–IV) activity by spectrophotometric methods. Results are presented as either (a) raw rates, or (b) relative to CS activity. Median is displayed for controls with error bars showing the 1.25th and 98.75th percentiles of the reference range, and the green shading region shows the range. Each data point represents the average of each patient or control from ≥ 2 technical replicates Abbreviations: CI–IV, respiratory chain complexes I–IV; CS, citrate synthase.