Figure 4.

Complexome Profiling of Fibroblasts from Subjects 1–3 Identifies Stalled Complex I Assembly Intermediates

Control (A), subject 1 (B), subject 2 (C), and subject 3 (D). The left lane indicates assembly factors (gray) and structural subunits (orange-brown) of complex I modules, complex III (red), and complex IV (green). Dashed boxes indicate the loss or reduction of N-module subunits in subject fibroblast lines. White arrows highlight the loss or reduction of NDUFA6, NDUFA7 and NDUFA12 from complex I in subjects 1–3, and blue arrows indicate the location of NDUFAF2, still bound to the stalled complex I intermediate in subject complexomes but not in the control. Assignment of complex I subunits to modules was according to Wirth et al.²⁵ Intensity-based absolute quantification (iBAQ) values were normalized to the sum of all values of the control. Native mass calibration was performed according to Fuhrmann et al.²⁶