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CHCHD4 Antibody

  • 货号：
    CSB-PA005319GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q8N4Q1
  • 基因名：
    CHCHD4
  • 别名：
    CHCHD4 antibody; MIA40Mitochondrial intermembrane space import and assembly protein 40 antibody; Coiled-coil-helix-coiled-coil-helix domain-containing protein 4 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human CHCHD4
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC,IF
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    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.
  • 基因功能参考文献：
    1. First group to discover the expression of the human CHCHD4 isoforms and first to call the human gene CHCHD4, and to clone and show the expression of its alternatively expressed CHCHD4 isoforms (CHCHD4.1 and CHCHD4.2). PMID: 22214851
    2. Compared to wild type control littermates, mice with a knockout of CHCHD4 exhibit reduced weight gain when fed a high-fat diet. PMID: 26178476
    3. Data show that the redox state of cytochrome c oxidase assembly protein 17 (Cox17), mitochondrial membrane transport protein Mia40 and superoxide dismutase 1 (SOD1) in the cytoplasm were directly observed with in-cell NMR spectroscopy. PMID: 26589182
    4. our findings suggest that MIA40 reduction contributes to the effects of AIF deficiency on OXPHOS, as it may impact on the correct assembly and maintenance of the respiratory subunits. PMID: 26158520
    5. results demonstrate an indispensable role for hMIA40 for the export of Fe-S clusters from mitochondria. PMID: 26275620
    6. Data indicate that poptosis-inducing factor (AIF) controls the mitochondrial import of mitochondrial membrane transport protein CHCHD4. PMID: 26004228
    7. In aggregate these data suggest that the Mia40/lfALR system has a broad sequence specificity and that potential substrates may be protected from adventitious oxidation by kinetic sequestration within the mitochondrial IMS. PMID: 26014136
    8. Import and oxidative folding of proteins are kinetically and functionally coupled and depend on the expression of Mia40, ALR, and the intracellular glutathione pool. PMID: 23676665
    9. Data indicate that decreased CHCHD4 expression prevents the mitochondrial translocation of p53 while augmenting its nuclear localization and activity. PMID: 24101517
    10. illustrate a very atypical behaviour for the Mia40 precursor compared to other substrates of the MIA pathway. By contrast, interaction with Erv1 occurs after 5 min of import and relies on a more stringent specificity PMID: 23937629
    11. mitochondrial localization of MIA40 requires sulfhydryl oxidase ALR in heterologous expression yeast system. PMID: 23186364
    12. Molecular recognition and substrate mimicry drive the electron-transfer process between MIA40 and ALR. PMID: 21383138
    13. biogenesis and function of MIA40 in the mitochondrial intermembrane space is dependent on redox processes involving conserved cysteine residues PMID: 16185709
    14. After passage across the translocase of the mitochondrial outer membrane Erv1 interacts via disulfide bonds with Mia40. PMID: 17336303
    15. analysis of how mitochondrial biogenesis switches the sorting pathway of the intermembrane space receptor Mia40 PMID: 18779329
    16. Catalysis involves a flow of reducing equivalents from the reduced CxC cysteine motif of Mia40 to distal and then proximal CxxC motifs of long-form ALR to the flavin ring and, finally, to cytochrome c or molecular oxygen. PMID: 19397338

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  • 亚细胞定位：
    Mitochondrion intermembrane space.
  • 组织特异性：
    Expressed in all tissues tested, suggesting an ubiquitous expression.
  • 数据库链接：

    HGNC: 26467

    OMIM: 611077

    KEGG: hsa:131474

    STRING: 9606.ENSP00000295767

    UniGene: Hs.517815