Mitochondrial membranes from fibroblasts of control (WT) and patient fibroblasts (upper panels, A–G) and heart tissue from wild‐type (WT) and Uqcrh
−/− mice (lower panels, B–J) were analysed by complexome profiling and the appearance of individual complexes and superassemblies were quantified.
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A, B
The average of IBAQ values of all identified subunits from complexes III, I and IV (full data including all individual subunits shown in Fig
EV4).
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C, D
Molecular mass region used to quantify the appearance of individual and supercomplexes.
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E, H
Sum of all IBAQ values and relative distribution of complex III as individual dimer or assembled into supercomplexes and large supercomplexes.
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F, I
Sum of all IBAQ values and relative distribution of complex I as individual or assembled into supercomplexes and large supercomplexes.
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G, J
Sum of all IBAQ values and relative distribution of complex IV as individual or assembled into supercomplexes.
Data information: Assignment of complexes: I, complex I; III2, complex III dimer; IV, complex IV; S0, supercomplex containing complex I and a dimer of complex III; S1, supercomplex containing complex I, a dimer of complex III and 1 copy of complex IV, SXL, Supercomplex or Megacomplex containing I, III and higher signals also additional copies of IV.