Molecular weight (DA)
22kD
Immunogen
Synthesized peptide derived from human CHCHD4 AA range: 35-85
Specificity
This antibody detects endogenous levels of CHCHD4 at Human/Mouse/Rat
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.03% sodium azide.
Dilution rate
WB 1:500-2000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Background
CHCHD4, a component of human mitochondria, belongs to a protein family whose members share 6 highly conserved cysteine residues constituting a -CXC-CX(9)C-CX(9)C- motif in the C terminus (Hofmann et al., 2005 [PubMed 16185709]).[supplied by OMIM, Mar 2008],
Function
domain:The CHCH domain contains a conserved twin Cys-X(9)-Cys motif which is required for import and stability of MIA40 in mitochondria.,function:Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Probably acts by forming a redox cycle with GFER/ERV1 that involves a disulfide relay system. Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of 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.,PTM:Forms intrachain disulfide bridges, but exists in different redox states.,similarity:Contains 1 CHCH domain.,subunit:Forms homooligomer.,tissue specificity:Ex
Other name
Mitochondrial intermembrane space import and assembly protein 40 (Coiled-coil-helix-coiled-coil-helix domain-containing protein 4)
Human protein sequence Database
Q8N4Q1
Mouse protein sequence database
Q8VEA4
Rat protein sequence database
Q5BJN5
Cellular localization
Mitochondrion intermembrane space .
Tissue expression
Expressed in all tissues tested, suggesting an ubiquitous expression.
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