Gene Symbol | CHCHD4 |
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Entrez Gene | 131474 |
Alt Symbol | MIA40, TIMM40 |
Species | Human |
Gene Type | protein-coding |
Description | coiled-coil-helix-coiled-coil-helix domain containing 4 |
Other Description | 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 |
Swissprots | Q96MY6 Q96AI2 Q8N4Q1 A8K3Z9 |
Accessions | EAW64180 EAW64181 EAW64182 EAW64183 Q8N4Q1 AK056271 BAB71132 AK290764 BAF83453 BC017082 AAH17082 BC033775 AAH33775 DB452249 DB501565 NM_001098502 NP_001091972 NM_144636 NP_653237 |
Function | 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. {ECO:0000269|PubMed:16185709, ECO:0000269|PubMed:19182799, ECO:0000269|PubMed:21059946, ECO:0000269|PubMed:23186364}. |
Subcellular Location | Mitochondrion intermembrane space {ECO:0000269|PubMed:16185709, ECO:0000269|PubMed:23186364}. |
Tissue Specificity | Expressed in all tissues tested, suggesting an ubiquitous expression. {ECO:0000269|PubMed:16185709}. |
Human MIA40 full length protein - ab165346 from Abcam
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MIA40 / CHCHD4 Antibody Blocking Peptide - LS-E1046 from LifeSpan Bioscience
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MIA40 / CHCHD4 Antibody Blocking Peptide - LS-E18620 from LifeSpan Bioscience
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Recombinant Human CHCHD4 Protein - H00131474-P01 from Novus Biologicals
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MIA40-Antibody-C-term-Blocking-peptide - BP11528b from Abgent, a WuXi AppTec company
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Human CHCHD4 protein - orb244920 from Biorbyt
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CHCHD4 Blocking Peptide - 33R-6527 from Fitzgerald
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CHCHD4 / MIA40 - H00131474-P01-10 from Acris Antibodies
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CHCHD4 / MIA40 - H00131474-P01-25 from Acris Antibodies
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CHCHD4 fusion protein - Ag15339 from Proteintech Group
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CHCHD4 fusion protein - ag15482 from Proteintech Group
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Recombinant Human Mitochondrial intermembrane space import and assembly protein - CSB-YP850802HU CSB-BP850802HU CSB-MP850802HU from Cusabio
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Recombinant human Mitochondrial intermembrane space import and assembly protein - CSB-EP850802HU from Cusabio
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CHCHD4 / MIA40 (transcript variant 1) - TP317831 from Acris Antibodies
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Recombinant Human CHCHD4 protein - CHCHD4-34H from Creative Biomart
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CHCHD4 Recombinant Protein Antigen - NBP1-83537PEP from Novus Biologicals
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CHCHD4 Peptide, Human - M34GP03067H from Creative Peptides
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CHCHD4 Recombinant Protein (OPCA02425) - OPCA02425 from Aviva Systems Biology
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Recombinant human Mitochondrial intermembrane space import and assembly protein 40 - AP71472 from Signalway Antibody
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CHCHD4 Recombinant Protein Antigen - NBP2-57060PEP from Novus Biologicals
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Mitochondrial intermembrane space import and assembly protein 40, Recombinant Protein - MBS9421336 from MyBioSource
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Mitochondrial intermembrane space import and assembly protein 40, Recombinant Protein - MBS1475488 from MyBioSource
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Recombinant Human CHCHD4 Protein (1-142 aa), His-SUMO-tagged - CHCHD4-1097H from Creative Biomart
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