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  4. BFSP2 Antibody

BFSP2 Antibody

  • 中文名稱：
    BFSP2兔多克隆抗體
  • 貨號：
    CSB-PA615697LA01HU
  • 規格：
    ￥440
  • 圖片：
    • Western Blot
      Positive WB detected in: Hela whole cell lysate, MCF-7 whole cell lysate, HepG2 whole cell lysate
      All lanes: BFSP2 antibody at 3.8µg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 46 kDa
      Observed band size: 46 kDa
    • IHC image of CSB-PA615697LA01HU diluted at 1:400 and staining in paraffin-embedded human eye 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 biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • Immunofluorescence staining of HepG2 cells with CSB-PA615697LA01HU at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) BFSP2 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    BFSP2
  • 別名：
    BFSP2Phakinin antibody; 49 kDa cytoskeletal protein antibody; Beaded filament structural protein 2 antibody; Lens fiber cell beaded filament protein CP 47 antibody; CP47 antibody; Lens fiber cell beaded filament protein CP 49 antibody; CP49 antibody; Lens intermediate filament-like light antibody; LIFL-L antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Phakinin protein (11-110AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated

    本頁面中的產品，BFSP2 Antibody (CSB-PA615697LA01HU)，的標記方式是Non-conjugated。對于BFSP2 Antibody，我們還提供其他標記。見下表：

    可提供標記
    標記方式 貨號 產品名稱 應用
    HRP CSB-PA615697LB01HU BFSP2 Antibody, HRP conjugated ELISA
    FITC CSB-PA615697LC01HU BFSP2 Antibody, FITC conjugated
    Biotin CSB-PA615697LD01HU BFSP2 Antibody, Biotin conjugated ELISA
  • 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, WB, IHC, IF
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:200-1:500
    IF 1:50-1:200
  • 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：
    Required for the correct formation of lens intermediate filaments as part of a complex composed of BFSP1, BFSP2 and CRYAA. Plays a role in maintenance of retinal lens optical clarity.
  • 基因功能參考文獻：
    1. The results suggest that the N-terminal domain of CRYAA is required during in vitro complex formation with filensin and phakinin. PMID: 28935373
    2. We report a novel mutation (p.G112E) in the BFSP2 gene, underscoring the physiological importance of the beaded filament protein and supporting its role in human cataract formation. PMID: 24654948
    3. Data show that BFSP2 and PITX3, hitherto known to cause eye defects only in a dominant fashion, can also present recessively. PMID: 21836522
    4. The Y-sutural opacity in the lens might be the typical and earliest sign for cataract caused by the BFSP2 mutation. In addition, a myopia susceptibility locus in this region, which might also be associated with the mutation in BFSP2. PMID: 15570218
    5. Progressive isolated sutural cataract associated with deletion mutation of BFSP2 gene in Chinese pedigree. Physiological importance of beaded filament protein. Role of BFSP2 in human cataract formation. PMID: 17200662
    6. Progressive phenotype has provided more evidence for the heterogeneity of congenital cataract caused by BFSP2 mutations and for the important role BFSP2 plays in cataract formation. PMID: 17982427
    7. This is the first reported case of a congenital lamellar cataract phenotype associated with the mutation of Arg339His (P.R339H) in BFSP2. PMID: 18958306

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  • 相關疾病：
    Cataract 12, multiple types (CTRCT12)
  • 亞細胞定位：
    Cell membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm. Cytoplasm, cytoskeleton. Cytoplasm, cell cortex.
  • 蛋白家族：
    Intermediate filament family
  • 組織特異性：
    Lens.
  • 數據庫鏈接：

    HGNC: 1041

    OMIM: 603212

    KEGG: hsa:8419

    STRING: 9606.ENSP00000304987

    UniGene: Hs.659862



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