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  4. CLPB Antibody, HRP conjugated

CLPB Antibody, HRP conjugated

  • 中文名稱：
    CLPB兔多克隆抗體, HRP偶聯
  • 貨號：
    CSB-PA884428LB01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) CLPB Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    CLPB
  • 別名：
    Caseinolytic peptidase B antibody; Caseinolytic peptidase B protein homolog antibody; clpB antibody; ClpB caseinolytic peptidase B homolog (E. coli) antibody; ClpB caseinolytic peptidase B homolog antibody; CLPB_HUMAN antibody; FLJ13152 antibody; HSP78 antibody; SKD3 antibody; Suppressor of potassium transport defect 3 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Caseinolytic peptidase B protein homolog protein (1-350AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    HRP
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA
  • 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：
    May function as a regulatory ATPase and be related to secretion/protein trafficking process. Involved in mitochondrial-mediated antiviral innate immunity, activates RIG-I-mediated signal transduction and production of IFNB1 and proinflammatory cytokine IL6.
  • 基因功能參考文獻：
    1. The neonatal presentation of CLPB deficiency predicts the course of disease in later life, which is extremely important for counselling. PMID: 28687938
    2. Case Reports: bi-allelic CLPB mutations cause cataract, renal cysts, nephrocalcinosis and 3-methylglutaconic aciduria, a novel disorder of mitochondrial protein disaggregation. PMID: 25595726
    3. Disruption of CLPB is associated with congenital microcephaly, severe encephalopathy and 3-methylglutaconic aciduria PMID: 25650066
    4. ClpB can passively thread soluble denatured proteins. PMID: 25288401
    5. Mutations in CLPB define a syndrome with intellectual disability, congenital neutropenia, progressive brain atrophy, movement disorder, cataracts, and 3-methylglutaconic aciduria. PMID: 25597510
    6. CLPB is proposed to function as a mitochondrial chaperone involved in disaggregation of misfolded proteins, resulting from stress such as heat denaturation. PMID: 25597511
    7. ClpB-DnaK reactivated all aggregated fusion proteins with similar efficiency, without unfolding native domains, demonstrating that partial threading of the misfolded moiety is sufficient to solubilize aggregates. PMID: 18488042
    8. formation of the DnaK-ClpB bichaperone network is a three step process PMID: 19698713

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  • 相關疾病：
    3-methylglutaconic aciduria with cataracts, neurologic involvement and neutropenia (MEGCANN)
  • 亞細胞定位：
    Mitochondrion intermembrane space.
  • 蛋白家族：
    ClpA/ClpB family
  • 組織特異性：
    Widely expressed (at protein level). Expressed in fetal, as well as in adult tissues, with highest levels in adult brain, including thalamus, hippocampus, occipital cortex and parietal cortex. Low expression in granulocytes.
  • 數據庫鏈接：

    HGNC: 30664

    OMIM: 616254

    KEGG: hsa:81570

    STRING: 9606.ENSP00000294053

    UniGene: Hs.523877



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