Product nameAnti-Bestrophin/BEST1 antibody
See all Bestrophin/BEST1 primary antibodies
DescriptionRabbit polyclonal to Bestrophin/BEST1
Tested applicationsSuitable for: WB, IHC-Pmore details
Species reactivityReacts with: Human
Recombinant fragment corresponding to Human Bestrophin/BEST1 aa 356-585.
Database link: O76090
- NT2D1, PC3 and U-87 MG whole cell lysates; Human U87 xenograft tissue.
This product was previously labelled as Bestrophin
Storage instructionsShipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Storage bufferpH: 7.00
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 78% PBS, 1% BSA, 20% Glycerol
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab155252 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/500 - 1/3000. Predicted molecular weight: 68 kDa.|
|IHC-P||1/100 - 1/1000.|
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
All lanes : Anti-Bestrophin/BEST1 antibody (ab155252) at 1/1000 dilution
Lane 1 : NT2D1 whole cell lysate
Lane 2 : PC3 whole cell lysate
Lane 3 : U-87 MG whole cell lysate
Lysates/proteins at 30 µg per lane.
Predicted band size: 68 kDa
7.5% SDS PAGE
Immunohistochemical analysis of paraffin-embedded Human U87 xenograft tissue labeling Bestrophin/BEST1 with ab155252 at 1/500 dilution.
ab155252 has not yet been referenced specifically in any publications.