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Neuroscience Cell Adhesion Proteins ECM Proteins
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Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)

  • Datasheet
  • SDS
Reviews (7)Q&A (1)References (22)

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Western blot - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)
  • Western blot - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)

Key features and details

  • Mouse monoclonal [E6-6] to Bestrophin/BEST1
  • Suitable for: WB
  • Reacts with: Human
  • Isotype: IgG1

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Overview

  • Product name

    Anti-Bestrophin/BEST1 antibody [E6-6]
    See all Bestrophin/BEST1 primary antibodies
  • Description

    Mouse monoclonal [E6-6] to Bestrophin/BEST1
  • Host species

    Mouse
  • Tested Applications & Species

    Application Species
    WB
    Human
    See all applications and species data
  • Immunogen

    Synthetic peptide corresponding to Human Bestrophin/BEST1 aa 568-585 (C terminal) conjugated to keyhole limpet haemocyanin.
    Sequence:

    KDHMDPYWALENRDEAHS


    Database link: O76090
    Run BLAST with BLAST the sequence with ExPASy Run BLAST with BLAST the sequence with NCBI
  • Positive control

    • IHC-Fr: Pig retinal pigment epithelium tissue. ICC/IF: Bovine retinal pigment epithelium (RPE). WB: Human RPE cell lysate.
  • General notes

     This product was previously labelled as Bestrophin

     This product has switched from ascites to TCS on 09th September 2020.

    The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing the problem with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation.

    One factor contributing to the crisis is the use of antibodies that are not suitable. This can lead to misleading results and the use of incorrect data informing project assumptions and direction. To help address this challenge, we have introduced an application and species grid on our primary antibody datasheets to make it easy to simplify identification of the right antibody for your needs.

    Learn more here.

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.
  • Storage buffer

    pH: 7.40
    Preservative: 0.1% Sodium azide
    Constituent: Ascites
  • Concentration information loading...
  • Purity

    Protein A/G purified
  • Clonality

    Monoclonal
  • Clone number

    E6-6
  • Myeloma

    unknown
  • Isotype

    IgG1
  • Light chain type

    kappa
  • Research areas

    • Neuroscience
    • Cell Adhesion Proteins
    • ECM Proteins
    • Neuroscience
    • Sensory System
    • Visual system

Associated products

  • Compatible Secondaries

    • Goat Anti-Mouse IgG H&L (Alexa Fluor® 488) (ab150113)
    • Goat Anti-Mouse IgG H&L (HRP) (ab205719)
  • Isotype control

    • Mouse IgG1, kappa monoclonal [15-6E10A7] - Isotype Control (ab170190)
  • Recombinant Protein

    • Recombinant Human Bestrophin/BEST1 protein (ab152797)

Applications

The Abpromise guarantee

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.

Guaranteed

Tested applications are guaranteed to work and covered by our Abpromise guarantee.

Predicted

Predicted to work for this combination of applications and species but not guaranteed.

Incompatible

Does not work for this combination of applications and species.

Application Species
WB
Human
All applications
Non human primates
Application Abreviews Notes
WB (3)
1/1000.
Notes
WB
1/1000.

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.
  • Target information above from: UniProt accession O76090 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt
  • Database links

    • Entrez Gene: 7439 Human
    • Omim: 607854 Human
    • SwissProt: O76090 Human
    • Unigene: 524910 Human
    • Unigene: 712676 Human
    • 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

    • Western blot - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)
      Western blot - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)
      Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182) at 1/1000 dilution + Human RPE cell lysate
    • Western blot - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)
      Western blot - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)This image is courtesy of an Abreview submitted by Dr Vladimir Milenkovic
      All lanes : Anti-Bestrophin/BEST1 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
      All lanes : 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


      The primary antobody was diluted in PBS/Tween/5%Milk and incubated for 1.5 hours at 25°C.

      See Abreview

    Protocols

    • Immunohistochemistry protocols
    • Western blot protocols

    Click here to view the general protocols

    Datasheets and documents

    • SDS download

    • Datasheet download

      Download

    References (22)

    Publishing research using ab2182? Please let us know so that we can cite the reference in this datasheet.

    ab2182 has been referenced in 22 publications.

    • Hazim RA  et al. Rapid differentiation of the human RPE cell line, ARPE-19, induced by nicotinamide. Exp Eye Res 179:18-24 (2019). PubMed: 30336127
    • Shen H  et al. A novel xeno-free culture system for human retinal pigment epithelium cells. Int J Ophthalmol 12:563-570 (2019). PubMed: 31024807
    • Wood SR  et al. A Quantitative Chloride Channel Conductance Assay for Efficacy Testing of AAV.BEST1. Hum Gene Ther Methods 30:44-52 (2019). PubMed: 30963787
    • Bai X  et al. Generation of an integration-free induced pluripotent stem cell line (FDEENTi003-A) from a patient with pathological myopia. Stem Cell Res 39:101495 (2019). PubMed: 31376721
    • Buskin A  et al. Disrupted alternative splicing for genes implicated in splicing and ciliogenesis causes PRPF31 retinitis pigmentosa. Nat Commun 9:4234 (2018). PubMed: 30315276
    View all Publications for this product

    Customer reviews and Q&As

    Show All Reviews Q&A
    Submit a review Submit a question

    1-8 of 8 Abreviews or Q&A

    Immunohistochemistry (Frozen sections) abreview for Anti-Bestrophin/BEST1 antibody [E6-6]

    Excellent
    Abreviews
    Abreviews
    abreview image
    Application
    Immunohistochemistry (Frozen sections)
    Sample
    Human Tissue sections (Eye, retinal pigment epithelium)
    Permeabilization
    Yes - 0.5% TX100
    Specification
    Eye, retinal pigment epithelium
    Blocking step
    Serum as blocking agent for 20 minute(s) · Concentration: 10% · Temperature: 25°C
    Fixative
    Paraformaldehyde
    Read More

    Herr Dr. Vladimir Milenkovic

    Verified customer

    Submitted May 29 2008

    Western blot abreview for Anti-Bestrophin/BEST1 antibody [E6-6]

    Excellent
    Abreviews
    Abreviews
    abreview image
    Application
    Western blot
    Sample
    Pig Cell lysate - other (Membrane fraction of the pig RPE cells)
    Gel Running Conditions
    Reduced Denaturing (10)
    Loading amount
    25 µg
    Specification
    Membrane fraction of the pig RPE cells
    Blocking step
    Milk as blocking agent for 30 minute(s) · Concentration: 5% · Temperature: 25°C
    Read More

    Herr Dr. Vladimir Milenkovic

    Verified customer

    Submitted Feb 25 2008

    Western blot abreview for Anti-Bestrophin/BEST1 antibody [E6-6]

    Good
    Abreviews
    Abreviews
    abreview image
    Application
    Western blot
    Sample
    Cow Cell lysate - whole cell (Retinal Pigment Epithelium cells)
    Gel Running Conditions
    Reduced Denaturing (10)
    Loading amount
    25 µg
    Specification
    Retinal Pigment Epithelium cells
    Blocking step
    Milk as blocking agent for 30 minute(s) · Concentration: 5% · Temperature: 25°C
    Read More

    Herr Dr. Vladimir Milenkovic

    Verified customer

    Submitted Feb 18 2008

    Immunocytochemistry/ Immunofluorescence abreview for Anti-Bestrophin/BEST1 antibody [E6-6]

    Excellent
    Abreviews
    Abreviews
    abreview image
    Application
    Immunocytochemistry/ Immunofluorescence
    Sample
    Cow Cell (Bovine Retinal Pigment Epithelium)
    Permeabilization
    Yes - 0.5% TX100
    Specification
    Bovine Retinal Pigment Epithelium
    Blocking step
    Serum as blocking agent for 20 minute(s) · Concentration: 10% · Temperature: 25°C
    Fixative
    Paraformaldehyde
    Read More

    Herr Dr. Vladimir Milenkovic

    Verified customer

    Submitted Feb 18 2008

    Western blot abreview for Anti-Bestrophin/BEST1 antibody [E6-6]

    Excellent
    Abreviews
    Abreviews
    abreview image
    Application
    Western blot
    Sample
    Human Cell lysate - other (RPE, retinal pigment epithelial cells)
    Gel Running Conditions
    Reduced Denaturing
    Loading amount
    20 µg
    Specification
    RPE, retinal pigment epithelial cells
    Blocking step
    Milk as blocking agent for 30 minute(s) · Concentration: 5% · Temperature: 25°C
    Read More

    Herr Dr. Vladimir Milenkovic

    Verified customer

    Submitted Jan 09 2008

    Immunohistochemistry (Frozen sections) abreview for Anti-Bestrophin/BEST1 antibody [E6-6]

    Excellent
    Abreviews
    Abreviews
    abreview image
    Application
    Immunohistochemistry (Frozen sections)
    Sample
    Pig Tissue sections (Retinal pigment epithelium)
    Permeabilization
    Yes - 0.5%TX100, 5% goat serum, 1XPBS
    Specification
    Retinal pigment epithelium
    Blocking step
    Serum as blocking agent for 20 minute(s) · Concentration: 5% · Temperature: 25°C
    Fixative
    Paraformaldehyde
    Read More

    Herr Dr. Vladimir Milenkovic

    Verified customer

    Submitted Jan 08 2008

    Immunoprecipitation abreview for Anti-Bestrophin/BEST1 antibody [E6-6]

    Excellent
    Abreviews
    Abreviews
    abreview image
    Application
    Immunoprecipitation
    Sample
    Human Cell lysate - whole cell (Expression of human Bestrophin 1in HEK 293 cells)
    Total protein in input
    20 µg
    Immuno-precipitation step
    Protein G
    Specification
    Expression of human Bestrophin 1in HEK 293 cells
    Read More

    Herr Dr. Vladimir Milenkovic

    Verified customer

    Submitted Jan 07 2008

    Question

    Hi, I've been trying to perform IHC on dog retinas using this Bestrophin Ab and am not having much success. The Ab works fine when using OCT embedded formaldehyde fixed retinas, but fails on Bouin fixed, paraffin embedded retinas. Are there any suggestions for a protocol to used with eyes fixed in this manner?

    Read More

    Abcam community

    Verified customer

    Asked on Apr 12 2006

    Answer

    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-embedded tissue sections. We have a nice general protocol for this that can found under the protocols section of the online datasheet for ab2182. Also, I did find some information on the IHCWorld website that "Bouin’s contains picric acid and any unbound picric acid left in the tissue will prevent adequate staining. It needs to be removed usually by washing the tissue well in running water before processing." You can also wash with 70% EtOH prior to processing. Otherwise, the acid will continue to negatively affect the tissue. Increased fixation does reduce permeability so you may need to reduce the fixation period. I hope this helps. I also encourage you to submit an Abreview for this antibody. We always encourage customers to feed their results back to us, whether positive or negative, and make all information about each of our products available to researchers. If you send us an Abreview, we can offer you 50 Abcam Points (a further 100 Abpoints will be offered for an image). To find out more about our Loyalty Scheme and Abpoints, please visit our website and look at Your Account panel (left bottom section on our home page) or the Abreviews section on the product datasheet. You can submit the Abreview if you click on the Abreview section (top menu bar) on the on-line datasheet of this antibody. It is a very straightforward process. Should you need any help how to submit an Abreview, then please do not hesitate to contact us again. Please contact us again if you have any additional questions.

    Read More

    Abcam Scientific Support

    Answered on Apr 13 2006

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