The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml. Predicted molecular weight: 16 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
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.
Zhuang J et al. Mitochondrial disulfide relay mediates translocation of p53 and partitions its subcellular activity. Proc Natl Acad Sci U S A110:17356-61 (2013).
Read more (PubMed: 24101517) »
Vascotto C et al. Knock-in reconstitution studies reveal an unexpected role of Cys-65 in regulating APE1/Ref-1 subcellular trafficking and function. Mol Biol Cell22:3887-3901 (2011).
Read more (PubMed: 21865600) »