Anti-Bestrophin/BEST1抗体[E6-6] (ab2182)
Key features and details
- Mouse monoclonal [E6-6] to Bestrophin/BEST1
- Suitable for: WB, IP, IHC-Fr, ICC/IF
- Reacts with: Cow, Dog, Human, Pig, Monkey
- Isotype: IgG1
概述
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产品名称
Anti-Bestrophin/BEST1抗体[E6-6]
参阅全部 Bestrophin/BEST1 一抗 -
描述
小鼠单克隆抗体[E6-6] to Bestrophin/BEST1 -
宿主
Mouse -
经测试应用
适用于: WB, IP, IHC-Fr, ICC/IFmore 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 -
免疫原
Synthetic peptide corresponding to Human Bestrophin/BEST1 aa 568-585 (C terminal) conjugated to keyhole limpet haemocyanin.
Sequence:KDHMDPYWALENRDEAHS
Database link: O76090 -
阳性对照
- IHC-Fr: Pig retinal pigment epithelium tissue. ICC/IF: Bovine retinal pigment epithelium (RPE). WB: Human RPE cell lysate.
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常规说明
This product was previously labelled as Bestrophin
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles. -
存储溶液
Preservative: 0.1% Sodium azide
Constituent: Ascites -
Concentration information loading... -
纯度
Ascites -
克隆
单克隆 -
克隆编号
E6-6 -
骨髓瘤
unknown -
同种型
IgG1 -
轻链类型
kappa -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
应用
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.
| 应用 | Ab评论 | 说明 |
|---|---|---|
| WB | 1/1000. | |
| IP | Use at an assay dependent concentration. | |
| IHC-Fr | Use at an assay dependent concentration. | |
| ICC/IF | 1/500. |
靶标
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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
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数据库链接
- Entrez Gene: 7439 Human
- Omim: 607854 Human
- SwissProt: O76090 Human
- SwissProt: Q8WMR7 Pig
- Unigene: 524910 Human
- Unigene: 712676 Human
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别名
- ARB antibody
- BEST 1 antibody
- BEST antibody
see all
图片
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![Immunohistochemistry (Frozen sections) - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)](https://www.abcam.cn/ps/products/2/ab2182/Images/ab2182-5453-anti-bestrophin-antibody-e6-6-immunohistochemistry.jpg)
Immunohistochemistry (Frozen sections) - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)This image is courtesy of an Abreview submitted by Dr Vladimir Milenkovicab2182 staining pig retinal pigment epithelium tissue sections. The tissue was fixed with paraformaldehyde, permeabilized in 0.5% TX100, 5% goat serum, 1XPBS, and blocked with 5% serum for 20 minutes at 25°C. The primary antibody was diluted 1/500 in 1% goat serum, 0.1% TX100, 1XPBS and incubated for 12 hours at 4°C. An Alexa Fluor® 488 conjugated goat anti-mouse was used as the secondary antibody.
The image shows bestrophin/BEST1, which localises to the retinal pigment epithelial (RPE) cells, labeled with green fluorescence. Nuclei were counterstained with DAPI (blue).
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![Western blot - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)](https://www.abcam.cn/ps/products/2/ab2182/Images/ab2182-5450-anti-bestrophin-antibody-e6-6-western-blot.jpg)
Western blot - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182) at 1/1000 dilution + Human RPE cell lysate -
![Immunocytochemistry/ Immunofluorescence - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)](https://www.abcam.cn/ps/products/2/ab2182/Images/ab2182-5451-anti-bestrophin-antibody-e6-6-immunofluorescence.jpg)
Immunocytochemistry/ Immunofluorescence - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)This image is courtesy of an Abreview submitted by Dr Vladimir Milenkovicab2182 staining cells of bovine retinal pigment epithelium (RPE) by ICC/IF. Cells were PFA fixed and permeabilized in 0.5% Triton X-100 prior to blocking in 10% goat serum for 20 minutes at 25°C. The primary antibody was diluted 1/500 and incubated with the sample for 12 hours at 4°C. An Alexa Fluor® 546 conjugated goat anti-mouse antibody was used as the secondary. Image A shows bestrophin/BEST1 localised in the basolateral side of the RPE cells labeled with red fluorescence (nuclei were counterstained with DAPI (blue)). Image B, a phase contrast (DIC) image, shows cellular structure.
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![Western blot - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)](https://www.abcam.cn/ps/products/2/ab2182/Images/ab2182-5452-anti-bestrophin-antibody-e6-6-western-blot.jpg)
Western blot - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)This image is courtesy of an Abreview submitted by Dr Vladimir MilenkovicAll 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 minutesThe primary antobody was diluted in PBS/Tween/5%Milk and incubated for 1.5 hours at 25°C.
实验方案
文献
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
