Your Good Partner in Biology Research

  1. Home
  2. 抗体
  3. 多克隆抗体
  4. BBS7 Antibody

BBS7 Antibody

  • 货号：
    CSB-PA814219LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • 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：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) BBS7 Polyclonal antibody
  • Uniprot No.：
    Q8IWZ6
  • 基因名：
    BBS7
  • 别名：
    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
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Bardet-Biedl syndrome 7 protein (289-393AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，BBS7 Antibody (CSB-PA814219LA01HU)，的标记方式是Non-conjugated。对于BBS7 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA814219LB01HU BBS7 Antibody, HRP conjugated ELISA
    FITC CSB-PA814219LC01HU BBS7 Antibody, FITC conjugated
    Biotin CSB-PA814219LD01HU BBS7 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IF 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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

    显示更多

    收起更多

  • 相关疾病：
    Bardet-Biedl syndrome 7 (BBS7)
  • 亚细胞定位：
    Cell projection, cilium membrane. Cytoplasm. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite. Cytoplasm, cytoskeleton, cilium basal body.
  • 组织特异性：
    Isoform 2 is ubiquitously expressed. Isoform 1 is expressed in retina, lung, liver, testis, ovary, prostate, small intestine, liver, brain, heart and pancreas.
  • 数据库链接：

    HGNC: 18758

    OMIM: 607590

    KEGG: hsa:55212

    STRING: 9606.ENSP00000264499

    UniGene: Hs.591694