Overview

  • Product name

  • Description

    Rabbit polyclonal to BBS1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-P, ICC/IFmore details
  • Species reactivity

    Reacts with: Mouse, Human
  • Immunogen

    Recombinant fragment corresponding to Human BBS1 aa 217-417.
    Database link: Q8NFJ9

  • Positive control

    • WB: Mouse lung tissue lysate. IHC: Human kidney and liver tissues. ICC/IF: HepG2 cells.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    pH: 7.40
    Preservative: 0.03% Proclin
    Constituents: 50% Glycerol, PBS
  • Concentration information loading...
  • Purity

    Protein G purified
  • Purification notes

    Purity >95%.
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab222890 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 1/500 - 1/5000. Detects a band of approximately 66 kDa (predicted molecular weight: 65 kDa).
IHC-P 1/20 - 1/200.
ICC/IF 1/50 - 1/200.

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 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

    Highly expressed in the kidney. Also found in fetal tissue, testis, retina, adipose tissue, heart, skeletal muscle and pancreas.
  • Involvement in disease

    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).
  • Cellular localization

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

  • Alternative names

    • 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

Images

  • HepG2 (human liver hepatocellular carcinoma cell line) cells stained for BBS1 (green) using ab222890 at 1/100 dilution in ICC/IF, followed by Alexa Fluor 488® conjugated Goat Anti-Rabbit IgG (H+L).

  • Paraffin-embedded human kidney tissue stained for BBS1 using ab222890 at 1/100 dilution in immunohistochemical analysis.

  • Paraffin-embedded human liver tissue stained for BBS1 using ab222890 at 1/100 dilution in immunohistochemical analysis.

  • Anti-BBS1 antibody (ab222890) at 1/500 dilution + Mouse lung lysate

    Secondary
    Goat polyclonal to rabbit IgG at 1/50000 dilution

    Predicted band size: 65 kDa
    Observed band size: 66 kDa
    why is the actual band size different from the predicted?

References

ab222890 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab222890.
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