Anti-Bestrophin/BEST1抗体[E6-6] (ab2182)
Key features and details
- Mouse monoclonal [E6-6] to Bestrophin/BEST1
- Suitable for: WB, IHC-Fr, ICC/IF, IP
- 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, IHC-Fr, ICC/IF, IPmore 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
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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 has switched from ascites to TCS on 9th September 2020.
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
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
pH: 7.40
Preservative: 0.02% Sodium azide
Constituent: 99% PBS -
Concentration information loading...
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纯度
Protein A/G purified -
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
应用 | Ab评论 | 说明 |
---|---|---|
WB | (3) |
1/1000.
|
IHC-Fr | (2) |
Use at an assay dependent concentration.
|
ICC/IF | (1) |
Use at an assay dependent concentration.
|
IP | (1) |
Use at an assay dependent concentration.
|
说明 |
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WB
1/1000. |
IHC-Fr
Use at an assay dependent concentration. |
ICC/IF
Use at an assay dependent concentration. |
IP
Use at an assay dependent concentration. |
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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Anti-Bestrophin/BEST1 antibody [E6-6] (ab2182) at 1/1000 dilution + Human RPE cell lysate
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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.
数据表及文件
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SDS download
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Datasheet download
文献 (30)
ab2182 被引用在 30 文献中.
- Asatryan A et al. New Retinal Pigment Epithelial Cell Model to Unravel Neuroprotection Sensors of Neurodegeneration in Retinal Disease. Front Neurosci 16:926629 (2022). PubMed: 35873810
- Senabouth A et al. Transcriptomic and proteomic retinal pigment epithelium signatures of age-related macular degeneration. Nat Commun 13:4233 (2022). PubMed: 35882847
- Zhang T et al. Determining the optimal stage for cryopreservation of human embryonic stem cell-derived retinal pigment epithelial cells. Stem Cell Res Ther 13:454 (2022). PubMed: 36064625
- Berber P et al. Transient Retention of Photoreceptor Outer Segments in Matrigel-Embedded Retinal Organoids. Int J Mol Sci 23:N/A (2022). PubMed: 36499228
- Afanasyeva TAV et al. A look into retinal organoids: methods, analytical techniques, and applications. Cell Mol Life Sci 78:6505-6532 (2021). PubMed: 34420069