Figure 2.

Protein factors modulating Mia40. AIF (Apoptosis-Inducing Factor) controls the import of CHCHD4 (Coiled-Coil-Helix-Coiled-Coil-Helix Domain Containing 4) in human cells. Gpx3 has an oxidising effect. Trx1 has a reducing effect as thioredoxins catalyse the protein disulphide exchange of proteins through a conserved active site. The navy double-headed arrow indicates a potential communication between these two systems. Erv1, and its human homologue ALR, have an oxidising effect on Mia40/CHCHD4 and are responsible for re-oxidising the CPC motif of Mia40 as part of the oxidative folding process in the IMS. Cells with mutated/knocked down Erv1 have reduced levels of MIA substrate import.