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Rather than causing a frank decrease in complex III function, this mutation appears to result in delayed assembly of the I,III,IV supercomplex, 43 thus influencing the interaction of complex III with other complexes rather than specifically affecting only complex III

doi:10.1001/jama.291.1.47 Mirer AG, Peppard PE, Palta M, Benca RM, Rasmuson A, Young T

It strongly depends on the oxidizing activity of Erv1 (Figure 7D), while at least at ambient oxygen concentrations (20%) complex IV activity appears to be dispensable (Figure 7E)