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MIA40 / CHCHD4

coiled-coil-helix-coiled-coil-helix domain containing 4

Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17. 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.

Gene Name: coiled-coil-helix-coiled-coil-helix domain containing 4
Synonyms: CHCHD4, CHDH4, TIMM40, MIA40
Target Sequences: Q8N4Q1 Q8N4Q1

Publications (1)

1
Rapid enrichment of mitochondria from mammalian cell cultures using digitonin. Bhavna Dixit, Shon Vanhoozer, Nana Abena Anti, Matthew S O'Connor, Amutha Boominathan. MethodsX. 2020 Dec;8:101197. [Full Text Article] [PubMed:34434723] [PMC:PMC8374178] Related Antibodies: LS-C802857.

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Proteins (5)
Over-Expression Lysate (2)
Recombinant (3)
MIA40 / CHCHD4 (5)
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GST (1)
His-SUMO (1)
Myc-DDK (Flag) (3)
Human (4)
293T Cells (1)
E. coli (1)
HEK 293 Cells (2)
Wheat Germ Extract (1)
Purified (1)
MIA40 / CHCHD4 Protein
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E. coli
His-SUMO
32 kD
1 mg/$1,355; 100 µg/$420; 20 µg/$323
MIA40 / CHCHD4 Protein - 12.5% SDS-PAGE of human CHCHD4 stained with Coomassie Blue
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Wheat Germ Extract
GST
42.4 kDa
10 µg/$479; 25 µg/$670
MIA40 / CHCHD4 Protein - Purified recombinant protein CHCHD4 was analyzed by SDS-PAGE gel and Coomassie Blue Staining
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HEK 293 Cells
Myc-DDK (Flag)
15.8 kDa
20 µg/$1,107
MIA40 / CHCHD4 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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293T Cells
Myc-DDK (Flag)
15.8 kDa
20 µg/$150
MIA40 / CHCHD4 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
15.8 kDa
100 µg/$494
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PLEASE NOTE

For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).