概述

  • 产品名称Anti-BBS1抗体
    参阅全部 BBS1 一抗
  • 描述
    兔多克隆抗体to BBS1
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Mouse
    预测可用于: Rat, Horse, Guinea pig, Cow, Cat, Dog, Pig
  • 免疫原

    Synthetic peptide corresponding to a region within N terminal amino acids 110-159 (PCVYVYKNLR PYFKFSLPQL PPNPLEQDVW NQAKEDQIDP LTLKEMLEDI) of Mouse BBS1 (NP_001028300).

  • 阳性对照
    • Mouse Liver Lysate.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液Constituents: 98% PBS, 2% Sucrose
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab111847 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 0.2 - 1 µg/ml. Predicted molecular weight: 65 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

靶标

  • 功能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 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.
  • 组织特异性Highly expressed in the kidney. Also found in fetal tissue, testis, retina, adipose tissue, heart, skeletal muscle and pancreas.
  • 疾病相关Defects in BBS1 are a cause of Bardet-Biedl syndrome type 1 (BBS1) [MIM:209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. A relatively high incidence of BBS is found in the mixed Arab populations of Kuwait and in Bedouin tribes throughout the Middle East, most likely due to the high rate of consaguinity in these populations and a founder effect. Inheritance is autosomal recessive, but three mutated alleles (two at one locus, and a third at a second locus) may be required for disease manifestation in some cases (triallelic inheritance).
  • 细胞定位Cell projection > cilium membrane. Cytoplasm. Localizes to nonmembranous centriolar satellites in the cytoplasm.
  • Information by UniProt
  • 数据库链接
  • 别名
    • AI451249 antibody
    • Bardet-Biedl syndrome 1 antibody
    • Bardet-Biedl syndrome 1 homolog antibody
    • Bardet-Biedl syndrome 1 protein antibody
    • BBS1 antibody
    • BBS1_HUMAN antibody
    • BBS2-like protein 2 antibody
    • BBS2L2 antibody
    • D19Ertd609e antibody
    see all

Anti-BBS1 antibody 图像

  • Anti-BBS1 antibody (ab111847) at 1 µg/ml + Mouse Liver Lysate at 10 µg

    Predicted band size : 65 kDa

Anti-BBS1 antibody (ab111847)参考文献

ab111847 has not yet been referenced specifically in any publications.

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