概述

  • 产品名称Anti-BBS7抗体
    参阅全部 BBS7 一抗
  • 描述
    小鼠单克隆抗体to BBS7
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Recombinant Fragment
    预测可用于: Human
  • 免疫原

    Recombinant fragment: SILKDVLSKE ATKRKINLNI SYEINEVSVK HTLKLIHPKL EYQLLLAKKV QLIDALKELQ IHEGNTNFLI PEYHCILEEA DHLQEEYKKQ PAHLERLYG, corresponding to amino acids 574-673 of Human BBS7

性能

应用

Our Abpromise guarantee covers the use of ab55023 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
  • 应用说明WB: Use at a concentration of 1-5 µg/ml.

    This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 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 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.
    • 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.
    • 疾病相关Note=Ciliary dysfunction leads to a broad spectrum of disorders, collectively termed ciliopathies. Overlapping clinical features include retinal degeneration, renal cystic disease, skeletal abnormalities, fibrosis of various organ, and a complex range of anatomical and functional defects of the central and peripheral nervous system. The ciliopathy range of diseases includes Meckel-Gruber syndrome, Bardet-Biedl syndrome, Joubert syndrome, nephronophtisis, Senior-Loken syndrome, and Jeune asphyxiating thoracic dystrophy among others. Single-locus allelism is insufficient to explain the variable penetrance and expressivity of such disorders, leading to the suggestion that variations across multiple sites of the ciliary proteome, including BBS7, influence the clinical outcome.
      Bardet-Biedl syndrome 7 (BBS7) [MIM:209900]: A syndrome 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. Bardet-Biedl syndrome inheritance is autosomal recessive, but three mutated alleles (two at one locus, and a third at a second locus) may be required for clinical manifestation of some forms of the disease. Note=The disease is caused by mutations affecting the gene represented in this entry.
    • 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
      • 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
      see all

    Anti-BBS7 antibody 图像

    • Western blot against tagged recombinant protein immunogen using ab55023 BBS7 antibody at 1ug/ml. Predicted band size of immunogen is 37 kDa.

      This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

    Anti-BBS7 antibody (ab55023)参考文献

    ab55023 has not yet been referenced specifically in any publications.

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