Overview

  • Product name
    Anti-Bestrophin antibody [E6-6]
    See all Bestrophin primary antibodies
  • Description
    Mouse monoclonal [E6-6] to Bestrophin
  • Specificity
    This antibody is specific for Bestrophin.
  • Tested applications
    Suitable for: WB, IP, IHC-Fr, ICC/IFmore details
  • Species reactivity
    Reacts with: Cow, Dog, Human, Pig, Monkey
    Does not react with: Mouse, Rat, Rabbit, Goat
  • Immunogen

    Recombinant fragment:

    KDHMDPYWALENRDEAHS

    , corresponding to C terminal amino acids 568-585 of Human Bestrophin.

  • Positive control
    • Pig RPE (Retinal Pigment Epithelium) whole cell extract (clone E6-1 or E6-6).

Properties

  • Form
    Liquid
  • Storage instructions
    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • Concentration information loading...
  • Purity
    Ascites
  • Purification notes
    Raw ascities containing 0.1% sodium azide.
  • Clonality
    Monoclonal
  • Clone number
    E6-6
  • Myeloma
    unknown
  • Isotype
    IgG1
  • Light chain type
    kappa
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab2182 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 1/1000.
IP Use at an assay dependent concentration.
IHC-Fr Use at an assay dependent concentration.
ICC/IF 1/500.

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

Anti-Bestrophin antibody [E6-6] images

  • ab2182 staining pig retinal pigment epithelium tissue sections.  The tissue was fixed with paraformaldehyde, permeabilised in 0.5% TX100, 5% goat serum, 1XPBS, and blocked with 5% serum for 20 minutes at 25°C.  The primary anitbody was diluted 1/500 in 1% goat serum, 0.1% TX100, 1XPBS and incubated for 12 hours at 4°C.   An Alexa Fluor® 488 conjugated goat anti-mouse was used as the secondary antibody.

    The image shows bestrophin, which localises to the retinal pigment epithelial (RPE) cells, labeled with green fluorescence. Nuclei were counterstained with DAPI (blue).

    See Abreview

  • All lanes : Anti-Bestrophin antibody [E6-6] (ab2182) at 1/1000 dilution

    Lane 1 : Human RPE, retinal pigment epithelial cell lysate
    Lane 2 : Non transfected HEK 293 cell extract

    Lysates/proteins at 20 µg per lane.

    Secondary
    HRP-conjugated goat anti-mouse
    Developed using the ECL technique

    Performed under reducing conditions.

    Observed band size : 67 kDa (why is the actual band size different from the predicted?)


    Exposure time : 5 minutes

    This image is courtesy of an Abreview submitted by Dr Vladimir Milenkovic

    See Abreview

  • ab2182 staining cells of bovine retinal pigment epithelium (RPE) by ICC/IF.  Cells were PFA fixed and permeabilized in 0.5% Triton X-100 prior to blocking in 10% goat serum for 20 minutes at 25°C.  The primary antibody was diluted 1/500 and incubated with the sample for 12 hours at 4°C.  An Alexa Fluor® 546 conjugated goat anti-mouse antibody was used as the secondary.  Image A shows bestrophin localized in the basolateral side of the RPE cells labeled with red fluorescence (nuclei were counterstained with DAPI (blue)).  Image B, a phase contrast (DIC) image, shows cellular structure.

    See Abreview

  • Western blot detection of Bestrophin (68 kDa)from human RPE cell lysate.

References for Anti-Bestrophin antibody [E6-6] (ab2182)

This product has been referenced in:
  • Samuel W  et al. Appropriately differentiated ARPE-19 cells regain phenotype and gene expression profiles similar to those of native RPE cells. Mol Vis 23:60-89 (2017). WB ; Human . Read more (PubMed: 28356702) »
  • Carter DA  et al. Mislocalisation of BEST1 in iPSC-derived retinal pigment epithelial cells from a family with autosomal dominant vitreoretinochoroidopathy (ADVIRC). Sci Rep 6:33792 (2016). Read more (PubMed: 27653836) »

See all 12 Publications for this product

Product Wall

Abcam guarantees this product to work in the species/application used in this Abreview.
Application
Immunohistochemistry (Frozen sections)
Sample
Human Tissue sections (Eye, retinal pigment epithelium)
Specification
Eye, retinal pigment epithelium
Fixative
Paraformaldehyde
Permeabilization
Yes - 0.5% TX100
Blocking step
Serum as blocking agent for 20 minute(s) · Concentration: 10% · Temperature: 25°C
Username

Herr Dr. Vladimir Milenkovic

Verified customer

Submitted May 29 2008

Abcam guarantees this product to work in the species/application used in this Abreview.
Application
Western blot
Sample
Pig Cell lysate - other (Membrane fraction of the pig RPE cells)
Loading amount
25 µg
Specification
Membrane fraction of the pig RPE cells
Gel Running Conditions
Reduced Denaturing (10)
Blocking step
Milk as blocking agent for 30 minute(s) · Concentration: 5% · Temperature: 25°C
Username

Herr Dr. Vladimir Milenkovic

Verified customer

Submitted Feb 25 2008

Abcam guarantees this product to work in the species/application used in this Abreview.
Application
Western blot
Sample
Cow Cell lysate - whole cell (Retinal Pigment Epithelium cells)
Loading amount
25 µg
Specification
Retinal Pigment Epithelium cells
Gel Running Conditions
Reduced Denaturing (10)
Blocking step
Milk as blocking agent for 30 minute(s) · Concentration: 5% · Temperature: 25°C
Username

Herr Dr. Vladimir Milenkovic

Verified customer

Submitted Feb 18 2008

Abcam guarantees this product to work in the species/application used in this Abreview.
Application
Immunocytochemistry/ Immunofluorescence
Sample
Cow Cell (Bovine Retinal Pigment Epithelium)
Specification
Bovine Retinal Pigment Epithelium
Fixative
Paraformaldehyde
Permeabilization
Yes - 0.5% TX100
Blocking step
Serum as blocking agent for 20 minute(s) · Concentration: 10% · Temperature: 25°C
Username

Herr Dr. Vladimir Milenkovic

Verified customer

Submitted Feb 18 2008

Abcam guarantees this product to work in the species/application used in this Abreview.
Application
Western blot
Sample
Human Cell lysate - other (RPE, retinal pigment epithelial cells)
Loading amount
20 µg
Specification
RPE, retinal pigment epithelial cells
Gel Running Conditions
Reduced Denaturing
Blocking step
Milk as blocking agent for 30 minute(s) · Concentration: 5% · Temperature: 25°C
Username

Herr Dr. Vladimir Milenkovic

Verified customer

Submitted Jan 09 2008

Abcam guarantees this product to work in the species/application used in this Abreview.
Application
Immunohistochemistry (Frozen sections)
Sample
Pig Tissue sections (Retinal pigment epithelium)
Specification
Retinal pigment epithelium
Fixative
Paraformaldehyde
Permeabilization
Yes - 0.5%TX100, 5% goat serum, 1XPBS
Blocking step
Serum as blocking agent for 20 minute(s) · Concentration: 5% · Temperature: 25°C
Username

Herr Dr. Vladimir Milenkovic

Verified customer

Submitted Jan 08 2008

Abcam guarantees this product to work in the species/application used in this Abreview.
Application
Immunoprecipitation
Sample
Human Cell lysate - whole cell (Expression of human Bestrophin 1in HEK 293 cells)
Total protein in input
20 µg
Specification
Expression of human Bestrophin 1in HEK 293 cells
Immuno-precipitation step
Protein G
Username

Herr Dr. Vladimir Milenkovic

Verified customer

Submitted Jan 07 2008

Thank you for your enquiry. Ab2182 has not previously been tested before for application in IHC so we don't have a protocol specific for this antibody. As you're having difficulty with the Bouin’s fixative, you may want to try formalin fixed paraffin-e...

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