Altered proteolytic processing of NDUFV1 and NDUFV2 in CLPP-deficient mice.A, Scheme of NDUFV1 with identified N termini indicated. MTS (yellow) and mature protein (blue) are shown with starting position of detected N termini. Above and below the protein, identified N-terminal peptides (underlined) are shown with the associated log2(Clpp−/−/wt) and the preceding sequence. Amino acids at the P1 position are highlighted in red. N termini with increased abundance are depicted in green, with decreased abundance in red and unchanged abundance in gray. B, Schemes of NDUFV2 with identified N termini using the same color scheme and predicted masses of the corresponding proteoforms. C, Immunoblot analysis of NDUFV2 with quantification of the corresponding bands. Differences between wild type and Clpp−/− were tested using a two-tailed Student's t test. *** indicates p-val <0.001.