Coiled coil helix coiled coil helix domain containing protein 4
Coiled-coil-helix-coiled-coil-helix domain-containing protein 4
Mitochondrial intermembrane space import and assembly 40 homolog
Mitochondrial intermembrane space import and assembly protein 40
Translocase of inner mitochondrial membrane 40 homolog
Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Probably acts by forming a redox cycle with GFER/ERV1 that involves a disulfide relay system. Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond and is able to undergo folding in the IMS.
Expressed in all tissues tested, suggesting an ubiquitous expression.
Contains 1 CHCH domain.
The CHCH domain contains a conserved twin Cys-X(9)-Cys motif which is required for import and stability of MIA40 in mitochondria.
Forms intrachain disulfide bridges, but exists in different redox states.