Supplementary Fig. 1 A Min vs Max %Mean Fibroblast Fibroblasts PDC Activity in PDHA1 Only Group PDHA1-Only Group Min vsSkeletal Max %MeanMuscle Skeletal Muscle PDC Activity in PDHA1Group Only Group PDHA1-Only C * 100 100 Discordance area 50 Female "Low" PDC cutoff (<52%) Male Max %mean Max %mean Discordance area * 50 Male Female "Low" PDC cutoff (<38%) 0 0 50 0 100 0 Min %mean Min vs Max %Mean Fibroblast All-CIDEM PDC Activity in All Group CIDEM Group B * 100 Discordance area Max %mean Max %mean Discordance area 50 Female "Low" PDC cutoff (<52%) Male 100 %mean Min vs MaxMin %Mean Skeletal Muscle PDC Activity in All CIDEM All-CIDEM GroupGroup D 100 50 ** * 50 Male Female "Low" PDC cutoff (<38%) 0 0 50 Min %mean 100 0 0 50 Min %mean 100 Supplementary Fig. 1. Discordance of successive repeat PDC testing in fibroblasts (A and B) and skeletal muscle (C and D). Plot of minimum vs maximum fibroblast PDC activity noted as % control mean (%mean) for the PDHA1-Only (A and C) and All-CIDEM (B and D) groups. “Low” PDC cutoff values (<52% and <38% for fibroblast and skeletal muscle assays, respectively and correspond to <3rd %ile of control range) noted as green lines along with the area of discordance (green box). See Materials and Methods Section 2.2 for control mean and range. The red and blue asterisks (C and D) represent values off the plot scale for the respective subjects; one female subject was considered discordant but was off scale. Y-axis (Max %mean) and X-axis (Min %mean) represent maximum and minimum PDC activities for a subject as % control mean, respectively.