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PEX7 Antibody

  • 货号：
    CSB-PA030038
  • 规格：
    ￥880
  • 图片：
    • Western Blot analysis of 3T3 cells using Peroxin 7 Polyclonal Antibody
  • 其他：

产品详情

  • Uniprot No.：
    O00628
  • 基因名：
    PEX7
  • 别名：
    PBD9B antibody; PCDP1 antibody; Peroxin 7 antibody; Peroxin-7 antibody; Peroxisomal PTS2 receptor antibody; Peroxisomal targeting signal 2 receptor antibody; Peroxisome biogenesis factor 7 antibody; Peroxisome targeting signal 2 receptor antibody; PEX7 antibody; PEX7 protein antibody; PEX7_HUMAN antibody; PTS2 receptor antibody; PTS2R antibody; RCDP1 antibody; RD antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Synthesized peptide derived from the Internal region of Human Peroxin 7.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, IHC, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    IHC 1:100-1:300
    ELISA 1:40000
  • 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：
    Binds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import.
  • 基因功能参考文献：
    1. This revealed a marked plasmalogen deficiency and a deficient fatty acid alpha-oxidation in the IHH cells, due to a defect of PEX7, a cytosolic receptor protein required for peroxisomal import of a subset of peroxisomal proteins. PMID: 28013369
    2. our data suggest that insertion of the trimeric PEX5-PEX7-PTS2 protein complex into the DTM is probably accompanied by conformational alterations in PEX5 to allow release of the PTS2 protein into the organelle matrix PMID: 26138649
    3. Mutation in the PEX7 Gene is associated with Rhizomelic Chondrodysplasia Punctata Type 1. PMID: 25800479
    4. the sequential formation of a highly stable trimeric complex involving cargo protein, PEX7 and PEX5L stabilizes cargo binding and is a prerequisite for PTS2-mediated peroxisomal import. PMID: 25538232
    5. dysfunctional Pex7p, including mutants from RCDP patients, is degraded by a ubiquitin-dependent proteasomal pathway involving the CRL4A (Cullin4A-RING ubiquitin ligase) complex. PMID: 24989250
    6. Export of peroxisomal PEX7 back into the cytosol requires export of PEX5. PMID: 24865970
    7. This results of this studt revealed the association of 2 single nucleotide polymorphisms and 1 haplotype with autism spectrum disorder (P < .05). PMID: 22378669
    8. Structural requirements for interaction of peroxisomal targeting signal 2 and its receptor PEX7. PMID: 22057399
    9. Mutations in PEX7 gene is associated with Rhizomelic chondrodysplasia punctata. PMID: 20145307
    10. mutational spectrum in the PEX7 gene of 78 patients (including five pairs of sibs) clinically and biochemically diagnosed with RCDP type I PMID: 11781871
    11. Functional studies on human Pex7p: subcellular localization and interaction with proteins containing a peroxisome-targeting signal type 2 and other peroxins. PMID: 11931631
    12. The residual activity of mutant Pex7 protein in rhizomelic chondrodysplasia punctata patients and reduced amounts of normal Pex7 are associated with milder and variant phenotypes. PMID: 12325024
    13. This gene codes for the peroxin 7 receptor protein required for peroxisomal import of proteins containing a peroxisomal targeting signal type 2. Mutations may result in a broad clinical spectrum of Refsum disease. PMID: 12522768
    14. Identification of PEX7 as the second gene involved in Refsum disease. PMID: 14713215
    15. Missense mutations, sequence duplications and deletions in PRX7 in 3 patients with the Refsum disease phenotypes. PMID: 14974078

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  • 相关疾病：
    Peroxisome biogenesis disorder complementation group 11 (PBD-CG11); Rhizomelic chondrodysplasia punctata 1 (RCDP1); Peroxisome biogenesis disorder 9B (PBD9B)
  • 亚细胞定位：
    Peroxisome. Cytoplasm.
  • 蛋白家族：
    WD repeat peroxin-7 family
  • 组织特异性：
    Ubiquitous. Highest expression in pancreas, skeletal muscle and heart.
  • 数据库链接：

    HGNC: 8860

    OMIM: 215100

    KEGG: hsa:5191

    STRING: 9606.ENSP00000315680

    UniGene: Hs.280932