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  4. CLPP Recombinant Monoclonal Antibody

CLPP Recombinant Monoclonal Antibody

  • 中文名稱：
    CLPP Recombinant Monoclonal Antibody
  • 貨號：
    CSB-RA128888A0HU
  • 規格：
    ￥1320
  • 圖片：
    • Western Blot
      Positive WB detected in: HEK293 whole cell lysate(30μg)
      All lanes: CLPP antibody at 1:1000
      Secondary
      Goat polyclonal to rabbit IgG at 1/40000 dilution
      Predicted band size: 30 kDa
      Observed band size: 30 kDa
      Exposure time：2min
    • IHC image of CSB-RA128888A0HU diluted at 1:100 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
    • IHC image of CSB-RA128888A0HU diluted at 1:100 and staining in paraffin-embedded human colorectal cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
    • Overlay Peak curve showing Raji cells stained with CSB-RA128888A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for?10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
  • 別名：
    ATP-dependent Clp protease proteolytic subunit, mitochondrial (EC 3.4.21.92) (Endopeptidase Clp), CLPP
  • 反應種屬：
    Human
  • 免疫原：
    A synthesized peptide derived from Human CLPP protein
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 克隆類型：
    Monoclonal
  • 抗體亞型：
    Rabbit IgG
  • 純化方式：
    Affinity-chromatography
  • 克隆號：
    19F6
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, WB, IHC, FC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:50-1:200
    FC 1:20-1:200
  • 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：
    Protease component of the Clp complex that cleaves peptides and various proteins in an ATP-dependent process. Has low peptidase activity in the absence of CLPX. The Clp complex can degrade CSN1S1, CSN2 and CSN3, as well as synthetic peptides (in vitro) and may be responsible for a fairly general and central housekeeping function rather than for the degradation of specific substrates. Cleaves PINK1 in the mitochondrion.
  • 基因功能參考文獻：
    1. Autophagy compensates impaired energy metabolism in CLPXP-deficient Podospora anserina strains and extends healthspan. PMID: 28449241
    2. we demonstrate that a strong mitochondrial cardiomyopathy and diminished respiration due to DARS2 deficiency can be alleviated by the loss of CLPP, leading to an increased de novo synthesis of individual OXPHOS subunits. PMID: 27154400
    3. ere we present eight families affected by Perrault syndrome. In five families we identified novel or previously reported variants in HSD17B4, LARS2, CLPP and C10orf2 PMID: 26970254
    4. Data suggest that tumors exploit ClpXP-directed proteostasis to maintain mitochondrial bioenergetics, buffer oxidative stress, and enable metastatic competence. PMID: 27389535
    5. Data indicate endopeptidase Clp (ClpP) mutation identified in two patients with Perrault syndrome type 3 in a Turkish family. PMID: 27087618
    6. ClpP proteases from E. coli, S. aureus, and human mitochondria exhibit preferences for certain amino acids in the P1, P2, and P3 positions . PMID: 26606371
    7. Optical trapping to assay single-molecule ClpXP unfolding and translocation of substrates consisting of domains with varying stabilities and sequences; find that ClpXP unfolds most domains by a single pathway, with kinetics that depend on the native fold and structural stability. PMID: 25083874
    8. Deletion of PaClpP, leads to an unexpected healthy phenotype and increased lifespan of the fungal ageing model organism Podospora anserina and This phenotype can be reverted by expression of human ClpP. PMID: 23360988
    9. Perrault syndrome is caused by recessive mutations in CLPP. PMID: 23541340
    10. the N-terminal peptide of ClpP is a structural component of the substrate translocation channel and may play an important functional role as well PMID: 15522782
    11. hClpX can regulate the appearance of hClpP peptidase activity in mitochondria and might affect the nature of the degradation products released during ATP-dependent proteolytic cycles PMID: 16115876
    12. We propose that decreased levels of mitochondrial proteases Lon and ClpP may allow heat shock protein 60 substrate proteins to go through more folding attempts PMID: 18378094

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  • 相關疾病：
    Perrault syndrome 3 (PRLTS3)
  • 亞細胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Peptidase S14 family
  • 組織特異性：
    Detected in liver (at protein level). Predominantly expressed in skeletal muscle. Intermediate levels in heart, liver and pancreas. Low in brain, placenta, lung and kidney.
  • 數據庫鏈接：

    HGNC: 2084

    OMIM: 601119

    KEGG: hsa:8192

    STRING: 9606.ENSP00000245816

    UniGene: Hs.515092



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