Knockdown of human GTPBP3 results in mitochondrial dysfunction. HEK-293 cells were transfected with GTPBP3 siRNAs or with negative control siRNAs, and oxygen consumption (A), superoxide detection in mitochondria with MitoSOX (B), mitochondrial membrane potential (C), and cellular ATP (D) were determined as specified in Materials and Methods. Bars indicate standard deviations of at least three different experiments (*, P < 0.05; **, P < 0.005; ***, P < 0.0005).