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. 2009 May 15;84(5):594–604. doi: 10.1016/j.ajhg.2009.04.004

Figure 6.

Figure 6

The Mitochondrial Disulfide Relay System

Schematic representation of the mitochondrial DRS, which mediates the import of cysteine-rich substrates into the IMS by an oxidative trapping mechanism. Mia40 performs the oxidation of these proteins and is reoxidized by Erv1/GFER in a disulfide-transfer reaction. For Erv1 to be recycled into its oxidized form, electrons are transferred to cytochrome c, connecting the DRS to the electron-transport chain of the mitochondria.