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PEX7 Antibody

  • 中文名稱:
    PEX7兔多克隆抗體
  • 貨號:
    CSB-PA017808GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
    PEX7
  • 別名:
    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
  • 反應種屬:
    Human,Mouse,Rat
  • 免疫原:
    Human PEX7
  • 免疫原種屬:
    Homo sapiens (Human)
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen Affinity purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,WB
  • Protocols:
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期:
    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.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能:
    Binds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import.
  • 基因功能參考文獻:
    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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  • 相關疾病:
    Peroxisome biogenesis disorder complementation group 11 (PBD-CG11); Rhizomelic chondrodysplasia punctata 1 (RCDP1); Peroxisome biogenesis disorder 9B (PBD9B)
  • 亞細胞定位:
    Peroxisome. Cytoplasm.
  • 蛋白家族:
    WD repeat peroxin-7 family
  • 組織特異性:
    Ubiquitous. Highest expression in pancreas, skeletal muscle and heart.
  • 數據庫鏈接:

    HGNC: 8860

    OMIM: 215100

    KEGG: hsa:5191

    STRING: 9606.ENSP00000315680

    UniGene: Hs.280932



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