Chchd4 (GeneName), Mitochondrial intermembrane space import and assembly protein 40 (ProteinName), MIA40_MOUSE.
Mouse Chchd4/ Mitochondrial intermembrane space import and assembly protein 40 ELISA Kit
Coiled-coil-helix-coiled-coil-helix domain-containing protein 4, Mia40
Natural and recombinant mouse Mitochondrial intermembrane space import and assembly protein 40
Serum, plasma, tissue homogenates, cell culture supernates and other biological fluids
Monomer. Can form homooligomers. Interacts with GFER and forms transient disulfide bonds with GFER. Interacts with MICU1.
Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17 or 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.
Mitochondrion intermembrane space
This product has not yet been referenced specifically in any publications.
"The Ca(2+)-Dependent Release of the Mia40-Induced MICU1-MICU2 Dimer from MCU Regulates Mitochondrial Ca(2+) Uptake."
"Protein import and oxidative folding in the mitochondrial intermembrane space of intact mammalian cells."
"Disulfide bond formation: sulfhydryl oxidase ALR controls mitochondrial biogenesis of human MIA40."
"Human CHCHD4 mitochondrial proteins regulate cellular oxygen consumption rate and metabolism and provide a critical role in hypoxia signaling and tumor progression."
"Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression."
"Molecular chaperone function of Mia40 triggers consecutive induced folding steps of the substrate in mitochondrial protein import."
"MIA40 is an oxidoreductase that catalyzes oxidative protein folding in mitochondria."
"Functional and mutational characterization of human MIA40 acting during import into the mitochondrial intermembrane space."
"Complete sequencing and characterization of 21,243 full-length human cDNAs."
"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
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