免疫原
Synthesized peptide derived from human CHCHD4 AA range: 35-85
特异性
This antibody detects endogenous levels of CHCHD4 at Human/Mouse/Rat
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.03% sodium azide.
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
背景（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],
功能
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
基因名称（Gene Name）
CHCHD4 MIA40
其他名称
Mitochondrial intermembrane space import and assembly protein 40 (Coiled-coil-helix-coiled-coil-helix domain-containing protein 4)
细胞定位
Mitochondrion intermembrane space .
组织表达
Expressed in all tissues tested, suggesting an ubiquitous expression.
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