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  4. SEC63 Antibody

SEC63 Antibody

  • 中文名稱：
    SEC63兔多克隆抗體
  • 貨號：
    CSB-PA020961GA01HU
  • 規格：
    ￥3,900
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
  • 別名：
    SEC63; SEC63L; Translocation protein SEC63 homolog
  • 宿主：
    Rabbit
  • 反應種屬：
    Human,Mouse,Rat
  • 免疫原：
    Human SEC63
  • 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 0.1% 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：
    Mediates cotranslational and post-translational transport of certain precursor polypeptides across endoplasmic reticulum (ER). Proposed to play an auxiliary role in recognition of precursors with short and apolar signal peptides. May cooperate with SEC62 and HSPA5/BiP to facilitate targeting of small presecretory proteins into the SEC61 channel-forming translocon complex, triggering channel opening for polypeptide translocation to the ER lumen. Required for efficient PKD1/Polycystin-1 biogenesis and trafficking to the plasma membrane of the primary cilia.
  • 基因功能參考文獻：
    1. Results suggest that loss of PRKCSH and SEC63 leads to general defects in ciliogenesis, while quenching of the Wnt signaling cascade is cholangiocyte-restricted. PMID: 28973524
    2. a SEC63 germline mutation may play a role in cyst formation in polycystic liver disease PMID: 23209713
    3. Phosphorylation of Sec63 by CK2 enhanced its binding to Sec62 PMID: 23287549
    4. Sec63 may perform a substrate-selective quantity control function during cotranslational endoplasmic reticulum import. PMID: 23166619
    5. Silencing the human SEC63 genes inhibits transport of only a subset of signal-peptide-containing precursor proteins to endoplasmic reticulum. PMID: 22375059
    6. identified nucleoredoxin as an interaction partner of Sec63; characterized this interaction; Sec63 is linked to the Wnt signaling pathways and this interaction may be the reason why mutations in SEC63 can lead to polycystic liver disease PMID: 21251912
    7. identified a total of 26 novel mutations in PRKCSH (n = 14) and SEC63 (n = 12), including four splice site mutations, eight insertions/ deletions, six non-sense mutations, and eight missense mutations PMID: 20095989
    8. Mutations in SEC63 cause autosomal dominant polycystic liver disease, suggesting a role for cotranslational protein-processing pathways in maintaining epithelial luminal structure and implicating noncilial ER proteins in human polycystic disease. PMID: 15133510
    9. Sec63p expression was observed in all cyst epithelia regardless of mutational state. And, Cystogenesis in SEC63-associated PCLD occurs via a different mechanism. PMID: 18224332

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  • 相關疾病：
    Polycystic liver disease 2 (PCLD2)
  • 亞細胞定位：
    Endoplasmic reticulum membrane; Multi-pass membrane protein.
  • 組織特異性：
    Widely expressed, with high levels in the liver.
  • 數據庫鏈接：

    HGNC: 21082

    OMIM: 608648

    KEGG: hsa:11231

    STRING: 9606.ENSP00000357998

    UniGene: Hs.26904