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TTC8 Antibody

  • 中文名称：
    TTC8兔多克隆抗体
  • 货号：
    CSB-PA025244GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q8TAM2
  • 基因名：
    TTC8
  • 别名：
    Bardet Biedl syndrome 8 protein antibody; Bardet Biedl syndrome type 8 antibody; Bardet-Biedl syndrome 8 protein antibody; BBS8 antibody; RP51 antibody; Tetratricopeptide repeat domain 8 antibody; Tetratricopeptide repeat protein 8 antibody; TPR repeat protein 8 antibody; TTC 8 antibody; Ttc8 antibody; TTC8_HUMAN antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human TTC8
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB
  • 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. A splice-site mutation in a retina-specific exon of TTC8 causes nonsyndromic retinitis pigmentosa. PMID: 20451172
    2. A homozygous null BBS8 mutation leads to Bardet-Biedl syndrome with randomization of left-right body axis symmetry, a known defect of the nodal cilium PMID: 14520415
  • 相关疾病：
    Retinitis pigmentosa 51 (RP51); Bardet-Biedl syndrome 8 (BBS8)
  • 亚细胞定位：
    Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cell projection, cilium membrane. Cytoplasm. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite. Cell projection, cilium.
  • 组织特异性：
    Widely expressed.
  • 数据库链接：

    HGNC: 20087

    OMIM: 608132

    KEGG: hsa:123016

    STRING: 9606.ENSP00000370031

    UniGene: Hs.303055