Key features and details
- Rabbit polyclonal to Bestrophin/BEST1
- Suitable for: IHC-P
- Reacts with: Human, Cynomolgus monkey
- Isotype: IgG
Product nameAnti-Bestrophin/BEST1 antibody
See all Bestrophin/BEST1 primary antibodies
DescriptionRabbit polyclonal to Bestrophin/BEST1
Tested applicationsSuitable for: IHC-Pmore details
Species reactivityReacts with: Human, Cynomolgus monkey
Synthetic peptide corresponding to Human Bestrophin/BEST1 aa 595-614 (C terminal).
- Conditioned medium cultured RPE cells (the basal expression of Bestrophin/BEST1 is low in cultured cells).
This product was previously labelled as Bestrophin
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab14929 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
FunctionForms calcium-sensitive chloride channels. Highly permeable to bicarbonate.
Tissue specificityPredominantly expressed in the basolateral membrane of the retinal pigment epithelium.
Involvement in diseaseDefects 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 similaritiesBelongs to the bestrophin family.
modificationsPhosphorylated by PP2A.
Cellular localizationCell membrane. Basolateral cell membrane.
- Information by UniProt
- ARB antibody
- BEST 1 antibody
- BEST antibody
IHC image of ab14929 staining in human normal hippocampus formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab14929, 5µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.
ab14929 has been referenced in 7 publications.
- Gu Q et al. Accreditation of Biosafe Clinical-Grade Human Embryonic Stem Cells According to Chinese Regulations. Stem Cell Reports 9:366-380 (2017). PubMed: 28506532
- Wu W et al. Features specific to retinal pigment epithelium cells derived from three-dimensional human embryonic stem cell cultures - a new donor for cell therapy. Oncotarget 7:22819-33 (2016). PubMed: 27009841
- Sohn EH et al. Allogenic iPSC-derived RPE cell transplants induce immune response in pigs: a pilot study. Sci Rep 5:11791 (2015). IHC-Fr . PubMed: 26138532
- Tucker BA et al. Patient-specific iPSC-derived photoreceptor precursor cells as a means to investigate retinitis pigmentosa. Elife 2:e00824 (2013). IHC . PubMed: 23991284
- 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
- Chen M et al. Characterization of a spontaneous mouse retinal pigment epithelial cell line B6-RPE07. Invest Ophthalmol Vis Sci 49:3699-706 (2008). WB ; Mouse . PubMed: 18421091
- Mullins RF et al. Differential macular and peripheral expression of bestrophin in human eyes and its implication for best disease. Invest Ophthalmol Vis Sci 48:3372-80 (2007). IHC ; Human . PubMed: 17591911