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BBS7 Antibody

  • 中文名稱：
    BBS7兔多克隆抗體
  • 貨號：
    CSB-PA814219LA01HU
  • 規(guī)格：
    ￥440
  • 圖片：
    • Immunofluorescence staining of Hela cells with CSB-PA814219LA01HU 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(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) BBS7 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    BBS7
  • 別名：
    Bardet Biedl syndrome 7 protein antibody; Bardet-Biedl syndrome 7 antibody; Bardet-Biedl syndrome 7 protein antibody; BBS2 like 1 antibody; BBS2 like protein 1 antibody; BBS2-like protein 1 antibody; BBS2L1 antibody; BBS7 antibody; BBS7_HUMAN antibody; FLJ10715 antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Bardet-Biedl syndrome 7 protein (289-393AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated

    本頁面中的產(chǎn)品，BBS7 Antibody (CSB-PA814219LA01HU)，的標(biāo)記方式是Non-conjugated。對于BBS7 Antibody，我們還提供其他標(biāo)記。見下表：

    可提供標(biāo)記
    標(biāo)記方式 貨號 產(chǎn)品名稱 應(yīng)用
    HRP CSB-PA814219LB01HU BBS7 Antibody, HRP conjugated ELISA
    FITC CSB-PA814219LC01HU BBS7 Antibody, FITC conjugated
    Biotin CSB-PA814219LD01HU BBS7 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(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, IF
  • 推薦稀釋比：
    Application Recommended Dilution
    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(chǎn)品評價

靶點詳情

  • 功能：
    The BBSome complex is thought to function as a coat complex required for sorting of specific membrane proteins to the primary cilia. The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane and binds to RAB3IP/Rabin8, the guanosyl exchange factor (GEF) for Rab8 and then the Rab8-GTP localizes to the cilium and promotes docking and fusion of carrier vesicles to the base of the ciliary membrane. The BBSome complex, together with the LTZL1, controls SMO ciliary trafficking and contributes to the sonic hedgehog (SHH) pathway regulation. Required for proper BBSome complex assembly and its ciliary localization.
  • 基因功能參考文獻：
    1. Two novel mutations and three previously reported variants, identified in the present study, further extend the body of evidence implicating BBS6, BBS7, BBS8, and BBS10 in causing Bardet-Biedl Syndrome. PMID: 28761321
    2. Sequence variants in BBS7 were identified in families with CRB2-related syndrome. PMID: 27004616
    3. BBS7 gene was a novel variant (c.103-1G>A) in the consensus splice acceptor site, which altered the splicing recognition site of 'AG' to 'AA' at the BBS7 gene intron 2 and exon 3 boundary. PMID: 25553308
    4. A novel Bardet-Biedl syndrome protein is identified anad characterized. PMID: 12567324
    5. This study describes a novel mutation in BBS7 causing Bardet-Biedl syndrome in a Chinese family. PMID: 19093007
    6. small role of BBS7 and TTC8 in the overall mutational load of Bardet-Biedl syndrome patients PMID: 19402160

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  • 相關(guān)疾病：
    Bardet-Biedl syndrome 7 (BBS7)
  • 亞細(xì)胞定位：
    Cell projection, cilium membrane. Cytoplasm. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite. Cytoplasm, cytoskeleton, cilium basal body.
  • 組織特異性：
    Isoform 2 is ubiquitously expressed. Isoform 1 is expressed in retina, lung, liver, testis, ovary, prostate, small intestine, liver, brain, heart and pancreas.
  • 數(shù)據(jù)庫鏈接：

    HGNC: 18758

    OMIM: 607590

    KEGG: hsa:55212

    STRING: 9606.ENSP00000264499

    UniGene: Hs.591694



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