Fig. 3: Analysis of mitochondrial OCR and EACR in control and patient fibroblast cells.

A Representative graph showing the OCR in control and P1 fibroblast cell lines. Injection of oligomycin, FCCP, and antimycin A and rotenone (A/R) are indicated (B) Representative graph showing the EACR in control and P1 cell lines. Injection of oligomycin, FCCP, and antimycin A and rotenone (A/R) are indicated (C) Mitochondrial complex I enzymatic activities in MRPS2 control and P1 cell lines (D) Mitochondrial complex IV enzymatic activities in control and P1 cell lines (E) Representative graph showing intracellular ATP levels. ATP determination kit was used to quantify intracellular ATP levels. *p < 0.05, **p < 0.001, ***p < 0.0001 and ****p < 0.00001.