Key features and details
- Mouse monoclonal [E6-6] to Bestrophin/BEST1
- Suitable for: WB
- Reacts with: Cow, Dog, Human, Pig, Monkey
- Isotype: IgG1
参阅全部 Bestrophin/BEST1 一抗
描述小鼠单克隆抗体[E6-6] to Bestrophin/BEST1
经测试应用适用于: WBmore details
种属反应性与反应: Cow, Dog, Human, Pig, Monkey
预测可用于: Non human primates不与反应: Mouse, Rat, Rabbit, Goat
- IHC-Fr: Pig retinal pigment epithelium tissue. ICC/IF: Bovine retinal pigment epithelium (RPE). WB: Human RPE cell lysate.
This product was previously labelled as Bestrophin
This product has switched from ascites to TCS on 09th September 2020.
存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Preservative: 0.1% Sodium azide
Concentration information loading...
纯度Protein A/G purified
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.
功能Forms calcium-sensitive chloride channels. Highly permeable to bicarbonate.
组织特异性Predominantly expressed in the basolateral membrane of the retinal pigment epithelium.
疾病相关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.
序列相似性Belongs to the bestrophin family.
翻译后修饰Phosphorylated by PP2A.
细胞定位Cell membrane. Basolateral cell membrane.
- Information by UniProt
- ARB antibody
- BEST 1 antibody
- BEST antibody
Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182) at 1/1000 dilution + Human RPE cell lysate
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.
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.
ab2182 被引用在 22 文献中.
- 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