Description
Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17, COX19 and MICU1. Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of CHCHD4/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. Reduced CHCHD4/MIA40 is then reoxidized by GFER/ERV1 via a disulfide relay system. Mediates formation of disulfide bond in MICU1 in the IMS, promoting formation of the MICU1-MICU2 heterodimer that regulates mitochondrial calcium uptake.
Sequence
MSYCRQEGKDRIIFVTKEDHETPSSAELVADDPNDPYEEHGLILPNGDINWNCPCLGGMASGPCGEQFKSAFSCFHYSTEDIKGSDCIDQFRAMQECMQKYPDLYPQDEEEEEEAKPVEPVEETADTKASAAKEQGASS
Simulated SDS-PAGE
![Western blot of Chchd4 recombinant protein](/recombinant/Chchd4-4.png)
(Note: Representative image - actual molecular weight may vary depending on tag type and expression method)
Safety
Upon ordering, we will perform rigorous biosecurity and export control screening to ensure that order fulfillment is consistent with all legal and regulatory guidance.
Protein synthesis service