Anti-Bestrophin/BEST1 antibody (ab14927)
Key features and details
- Rabbit polyclonal to Bestrophin/BEST1
- Suitable for: ICC, IP, ICC/IF, WB
- Reacts with: Mouse, Rat, Cow, Human
- Isotype: IgG
Overview
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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: ICC, IP, ICC/IF, WBmore details -
Species reactivity
Reacts with: Mouse, Rat, Cow, Human
Predicted to work with: Cynomolgus monkey -
Immunogen
Synthetic peptide corresponding to Human Bestrophin/BEST1 aa 1-100.
Database link: O76090 -
General notes
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 this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Properties
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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: 0.02% Sodium azide
Constituents: Tris glycine, 0.5% BSA, 30% Glycerol -
Concentration information loading...
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Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Recombinant Protein
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab14927 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 |
---|---|---|
ICC | (1) |
Use at an assay dependent concentration.
|
IP | (1) |
Use at an assay dependent concentration.
|
ICC/IF | (1) |
Use at an assay dependent concentration.
|
WB | (2) |
Use at an assay dependent concentration.
|
Notes |
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ICC
Use at an assay dependent concentration. |
IP
Use at an assay dependent concentration. |
ICC/IF
Use at an assay dependent concentration. |
WB
Use at an assay dependent concentration. |
Target
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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
modificationsPhosphorylated by PP2A. -
Cellular localization
Cell membrane. Basolateral cell membrane. - Information by UniProt
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Database links
- Entrez Gene: 7439 Human
- Entrez Gene: 24115 Mouse
- Entrez Gene: 293735 Rat
- Omim: 607854 Human
- SwissProt: O76090 Human
- SwissProt: O88870 Mouse
- Unigene: 524910 Human
- Unigene: 712676 Human
see all -
Alternative names
- ARB antibody
- BEST 1 antibody
- BEST antibody
see all
Protocols
Datasheets and documents
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SDS download
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Datasheet download
References (7)
ab14927 has been referenced in 7 publications.
- Zhang H et al. Transplantation of GMP-grade human iPSC-derived retinal pigment epithelial cells in rodent model: the first pre-clinical study for safety and efficacy in China. Ann Transl Med 9:245 (2021). PubMed: 33708872
- McCormick R et al. Optimisation of a Novel Bio-Substrate as a Treatment for Atrophic Age-Related Macular Degeneration. Front Bioeng Biotechnol 8:456 (2020). PubMed: 32500067
- Geng Z et al. Generation of retinal pigmented epithelium from iPSCs derived from the conjunctiva of donors with and without age related macular degeneration. PLoS One 12:e0173575 (2017). PubMed: 28282420
- Lin YH et al. Opening a New Time Window for Treatment of Stroke by Targeting HDAC2. J Neurosci 37:6712-6728 (2017). PubMed: 28592694
- Brennan SC et al. The extracellular calcium-sensing receptor regulates human fetal lung development via CFTR. Sci Rep 6:21975 (2016). PubMed: 26911344
- Guziewicz KE et al. Molecular consequences of BEST1 gene mutations in canine multifocal retinopathy predict functional implications for human bestrophinopathies. Invest Ophthalmol Vis Sci 52:4497-505 (2011). PubMed: 21498618
- Günzel D et al. Claudin-16 affects transcellular Cl- secretion in MDCK cells. J Physiol 587:3777-93 (2009). PubMed: 19528248