Host: |
Rabbit |
Applications: |
WB/IHC |
Reactivity: |
Human/Mouse/Rat |
Note: |
STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
Short Description: |
Rabbit polyclonal antibody anti-CHCHD4 (1-142) is suitable for use in Western Blot and Immunohistochemistry research applications. |
Clonality: |
Polyclonal |
Conjugation: |
Unconjugated |
Isotype: |
IgG |
Formulation: |
PBS with 0.01% Thimerosal, 50% Glycerol, pH7.3. |
Purification: |
Affinity purification |
Dilution Range: |
WB 1:500-1:2000IHC-P 1:100-1:200 |
Storage Instruction: |
Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
Gene Symbol: |
CHCHD4 |
Gene ID: |
131474 |
Uniprot ID: |
MIA40_HUMAN |
Immunogen Region: |
1-142 |
Immunogen: |
Recombinant fusion protein containing a sequence corresponding to amino acids 1-142 of human CHCHD4 (NP_001091972.1). |
Immunogen Sequence: |
MSYCRQEGKDRIIFVTKEDH ETPSSAELVADDPNDPYEEH GLILPNGNINWNCPCLGGMA SGPCGEQFKSAFSCFHYSTE EIKGSDCVDQFRAMQECMQK YPDLYPQEDEDEEEEREKKP AEQAEETAPIEATATKEEEG SS |
Tissue Specificity | Expressed in all tissues tested, suggesting an ubiquitous expression. |
Post Translational Modifications | Forms intrachain disulfide bridges, but exists in different redox states. |
Function | Central component of a redox-sensitive mitochondrial intermembrane space import machinery which is required for the biogenesis of respiratory chain complexes. Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17, COX19, MICU1 and COA7. Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Required for the import of COA7 in the 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. |
Protein Name | Mitochondrial Intermembrane Space Import And Assembly Protein 40Coiled-Coil-Helix-Coiled-Coil-Helix Domain-Containing Protein 4 |
Database Links | Reactome: R-HSA-1268020 |
Cellular Localisation | Mitochondrion Intermembrane Space |
Alternative Antibody Names | Anti-Mitochondrial Intermembrane Space Import And Assembly Protein 40 antibodyAnti-Coiled-Coil-Helix-Coiled-Coil-Helix Domain-Containing Protein 4 antibodyAnti-CHCHD4 antibodyAnti-MIA40 antibody |
Information sourced from Uniprot.org
12 months for antibodies. 6 months for ELISA Kits. Please see website T&Cs for further guidance