Overview

  • Product name

    Anti-Bestrophin/BEST1 antibody
    See all Bestrophin/BEST1 primary antibodies
  • Description

    Rabbit polyclonal to Bestrophin/BEST1
  • Host species

    Rabbit
  • Tested applications

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

    Reacts with: Rat, Human
    Predicted to work with: Cynomolgus monkey
  • Immunogen

    Synthetic peptide corresponding to Human Bestrophin/BEST1 aa 445-464.
    Sequence:

    WKLKAVDAFKSAPLYQRPGY

  • Positive control

    • Conditioned medium cultured RPE cells (the basal expression of Bestrophin/BEST1 is low in cultured cells).
  • General notes

     This product was previously labelled as Bestrophin

     

Properties

Applications

Our Abpromise guarantee covers the use of ab14928 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. Predicted molecular weight: 68 kDa.
ICC/IF Use at an assay dependent dilution.

Target

  • Function

    Forms calcium-sensitive chloride channels. Highly permeable to bicarbonate.
  • Tissue specificity

    Predominantly expressed in the basolateral membrane of the retinal pigment epithelium.
  • Involvement in disease

    Defects in BEST1 are the cause of vitelliform macular dystrophy type 2 (VMD2) [MIM:153700]; also known as Best macular dystrophy (BMD). VMD2 is an autosomal dominant form of macular degeneration that usually begins in childhood or adolescence. VMD2 is characterized by typical 'egg-yolk' macular lesions due to abnormal accumulation of lipofuscin within and beneath the retinal pigment epithelium cells. Progression of the disease leads to destruction of the retinal pigment epithelium and vision loss.
    Defects in BEST1 are the cause of retinitis pigmentosa type 50 (RP50) [MIM:613194]. A retinal dystrophy belonging to the group of pigmentary retinopathies. RP is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.
    Defects in BEST1 are a cause of adult-onset vitelliform macular dystrophy (AVMD) [MIM:608161]. AVMD is a rare autosomal dominant disorder with incomplete penetrance and highly variable expression. Patients usually become symptomatic in the fourth or fifth decade of life with a protracted disease of decreased visual acuity.
    Defects in BEST1 are the cause of bestrophinopathy autosomal recessive (ARB) [MIM:611809]. A retinopathy characterized by central visual loss, an absent electro-oculogram light rise, and a reduced electroretinogram.
    Defects in BEST1 are the cause of vitreoretinochoroidopathy autosomal dominant (ADVIRC) [MIM:193220]. A disorder characterized by vitreoretinochoroidal dystrophy. The clinical presentation is variable and may be associated with cataract, nanophthalmos, microcornea, shallow anterior chamber, and glaucoma.
  • Sequence similarities

    Belongs to the bestrophin family.
  • Post-translational
    modifications

    Phosphorylated by PP2A.
  • Cellular localization

    Cell membrane. Basolateral cell membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • ARB antibody
    • BEST 1 antibody
    • BEST antibody
    • Best disease antibody
    • Best macular dystrophy antibody
    • BEST1 antibody
    • BEST1_HUMAN antibody
    • Best1V1Delta2 antibody
    • Bestrophin 1 antibody
    • Bestrophin-1 antibody
    • Bestrophin1 antibody
    • BMD antibody
    • mBest1 antibody
    • RP50 antibody
    • TU15B antibody
    • Vitelliform macular dystrophy 2 antibody
    • Vitelliform macular dystrophy antibody
    • Vitelliform macular dystrophy protein 2 antibody
    • VMD 2 antibody
    • VMD2 antibody
    see all

Images

  • Immunofluorescence analysis of hESC-RPE monolayers, staining Bestrophin/BEST1 with ab14928.

    Cells were fixed with 4% paraformaldehyde, permeabilized with 0.1% Triton X-100 and blocked with 3% BSA for 1 hour. Cells were incubated with primary antibody (1/500) for 1 hour at room temperature. An AlexaFluor&reg568-conjugated goat anti-rabbit IgG (1/800) was used as the secondary antibody.

References

This product has been referenced in:

  • Chen CY  et al. N-Terminomics identifies HtrA1 cleavage of thrombospondin-1 with generation of a proangiogenic fragment in the polarized retinal pigment epithelial cell model of age-related macular degeneration. Matrix Biol N/A:N/A (2018). ICC/IF ; Human . Read more (PubMed: 29572155) »
  • Bennis A  et al. Stem Cell Derived Retinal Pigment Epithelium: The Role of Pigmentation as Maturation Marker and Gene Expression Profile Comparison with Human Endogenous Retinal Pigment Epithelium. Stem Cell Rev 13:659-669 (2017). ICC/IF ; Human . Read more (PubMed: 28730556) »
See all 4 Publications for this product

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