Overview

  • Product name

  • Description

    Goat polyclonal to BBS7
  • Host species

    Goat
  • Specificity

    ab156808 is expected to recognize both isoforms (NP_789794.1; NP_060660.2).
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Cow, Dog
  • Immunogen

    Synthetic peptide, corresponding to internal sequence amino acids 647-658 of Human BBS7 (NP_789794.1; NP_060660.2).

  • Positive control

    • Human skeletal muscle lysate.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • Storage buffer

    pH: 7.30
    Preservative: 0.02% Sodium azide
    Constituents: 99% Tris buffered saline, 0.5% BSA
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Purification notes

    ab156808 was purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab156808 in the following tested applications.

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

Application Abreviews Notes
WB Use a concentration of 1 - 3 µg/ml. Detects a band of approximately 80 kDa (predicted molecular weight: 80 kDa).

Target

  • Function

    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.
  • Tissue specificity

    Isoform 2 is ubiquitously expressed. Isoform 1 is expressed in retina, lung, liver, testis, ovary, prostate, small intestine, liver, brain, heart and pancreas.
  • Involvement in disease

    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.
  • Cellular localization

    Cell projection > cilium membrane. Cytoplasm. Localizes to nonmembranous centriolar satellites in the cytoplasm.
  • Information by UniProt
  • Database links

  • Alternative names

    • 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

Images

  • Anti-BBS7 antibody (ab156808) at 1 µg/ml + Human skeletal muscle lysate (in RIPA buffer) at 35 µg

    Developed using the ECL technique.

    Predicted band size: 80 kDa
    Observed band size: 80 kDa



    Primary incubation was 1 hour.

References

ab156808 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab156808.
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