Anti-Bestrophin/BEST1 抗体 [E6-6] (ab2182)
製品の概要
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製品名
Anti-Bestrophin/BEST1 antibody [E6-6]
Bestrophin/BEST1 一次抗体 製品一覧 -
製品の詳細
Mouse monoclonal [E6-6] to Bestrophin/BEST1 -
由来種
Mouse -
アプリケーション
適用あり: WB, IP, IHC-Fr, ICC/IFmore details -
種交差性
交差種: Cow, Dog, Human, Pig, Monkey
交差が予測される動物種: Non human primates
非交差種:
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 -
ポジティブ･コントロール
- IHC-Fr: Pig retinal pigment epithelium tissue. ICC/IF: Bovine retinal pigment epithelium (RPE). WB: Human RPE cell lysate.
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特記事項
This product was previously labelled as Bestrophin
製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles. -
バッファー
Preservative: 0.1% Sodium azide
Constituent: Ascites -
Concentration information loading... -
精製度
Ascites -
ポリ/モノ
モノクローナル -
クローン名
E6-6 -
ミエローマ
unknown -
アイソタイプ
IgG1 -
軽鎖の種類
kappa -
研究分野
関連製品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
アプリケーション
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.
| アプリケーション | Abreviews | 特記事項 |
|---|---|---|
| WB | 1/1000. | |
| IP | Use at an assay dependent concentration. | |
| IHC-Fr | Use at an assay dependent concentration. | |
| ICC/IF | 1/500. |
ターゲット情報
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機能
Forms calcium-sensitive chloride channels. Highly permeable to bicarbonate. -
組織特異性
Predominantly expressed in the basolateral membrane of the retinal pigment epithelium. -
関連疾患
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. -
配列類似性
Belongs to the bestrophin family. -
翻訳後修飾
Phosphorylated by PP2A. -
細胞内局在
Cell membrane. Basolateral cell membrane. - Information by UniProt
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参照データベース
- Entrez Gene: 7439 Human
- Omim: 607854 Human
- SwissProt: O76090 Human
- SwissProt: Q8WMR7 Pig
- Unigene: 524910 Human
- Unigene: 712676 Human
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別名
- ARB antibody
- BEST 1 antibody
- BEST antibody
see all
画像
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![Immunohistochemistry (Frozen sections) - Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182)](https://www.abcam.co.jp/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.co.jp/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.co.jp/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.co.jp/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.
プロトコール
参考文献
ab2182 は 22 報の論文で使用されています。
- 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
