Mitochondrial functional and regulatory proteins and markers of mitochondrial biogenesis are increased in KLF1^+/E325K cells. (A) Z-scored scaled abundance values for (i) mitochondrial proteins (GO:0005739) and (ii) proteins of the respiratory electron transport chain (GO:0022904) in WT and KLF1^+/E325K cells. (B) Heat maps of z-scored abundance values for respiratory complex I, II, III, and IV, adenosine triphosphate synthase, and MICOS complex proteins in WT and KLF1^+/E325K cells. Each box represents 1 biological repeat (total n = 3). (C) Scaled abundance of proteins encoded by the mitochondrial genome, (D) mitochondrial transcription factor complex components, and (E) catalytic/accessory subunits of mitochondrial DNA polymerase. All mean ± SD; n = 3; ∗P < .05, ∗∗P < .01, and ∗∗∗P < .001 compared with that for WT. (F) Markers of mitochondrial biogenesis: (i) cardiolipin concentration, (ii) mitochondrial-to-nuclear DNA ratio (determined via the qPCR analysis of mitochondrial genes ND1 and TL1, nuclear gene MSGN1 and an intergenic region; for primers, see Supplementary Table 3) in WT, KLF1^+/E325K, and KLF1^+/−cells, and (iii) scaled abundance of citrate synthase in WT and KLF1^+/E325K cells. (G) Scaled abundance of mitochondrial inner membrane scaffolding protein, PHB2; mean ± SD, n = 3; ∗∗∗P < .001 compared with WT. (H) Scaled abundance of phosphatase and tensin homolog (PTEN). Abundance values were from WT and KLF1^+/E325K day 2 of differentiation comparative proteomic data set.