Figure 3:

A-C Results of experiments on fibroblasts assessed on a Seahorse Bioscience XF24 Flux Analyzer. A. Oxygen Consumption Rate (OCR) at baseline B. Extracellular Acidification Rate (ECAR) at baseline. C. Spare Respiratory Capacity (defined as maximal respiration minus basal respiration). Data was analyzed using XF Cell Mito Stress Test Report Generator. Values were normalized per ug/ul of protein. Unpaired t-test with unequal variance was performed for statistical analysis (mean +/− SEM). D-E Results of experiments on fibroblast protein lysates assessed using SDS-Page and applicable antibodies D. anti-NDUFAF5, anti-NDUFS3 and anti-TOMM20 with anti-beta-Actin as the loading control. Quantification of horizontal slices was performed using Image J and displayed as % of control, Images show cropped bands. E. Oxphos antibody cocktail interrogating stability of complex I with anti-NDUFB8, complex II with anti-SDHB, complex III with anti-UQCRC2, complex IV with anti-COX II, and complex V with anti-ATP5A, anti-beta-Actin was used as the loading control. Image was cropped for beta Actin only. Quantification of data displayed as % of control. Striped shading indicates patient 2, black shading patient 3.