Anti-Bestrophin/BEST1 抗体 (ab14927)
Key features and details
- Rabbit polyclonal to Bestrophin/BEST1
- Suitable for: ICC, IP, ICC/IF, WB
- Reacts with: Mouse, Rat, Cow, Human
- Isotype: IgG
製品の概要
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製品名
Anti-Bestrophin/BEST1 antibody
Bestrophin/BEST1 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to Bestrophin/BEST1 -
由来種
Rabbit -
アプリケーション
適用あり: ICC, IP, ICC/IF, WBmore details -
種交差性
交差種: Mouse, Rat, Cow, Human
交差が予測される動物種: Cynomolgus monkey
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免疫原
Synthetic peptide corresponding to Human Bestrophin/BEST1 aa 1-100.
Database link: O76090 -
特記事項
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
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
pH: 7.40
Preservative: 0.02% Sodium azide
Constituents: Tris glycine, 0.5% BSA, 30% Glycerol -
Concentration information loading... -
精製度
Immunogen affinity purified -
ポリ/モノ
ポリクローナル -
アイソタイプ
IgG -
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
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab14927の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
| アプリケーション | Abreviews | 特記事項 |
|---|---|---|
| ICC | (1) |
Use at an assay dependent concentration.
|
| IP | (1) |
Use at an assay dependent concentration.
|
| ICC/IF | (1) |
Use at an assay dependent concentration.
|
| WB | (2) |
Use at an assay dependent concentration.
|
| 特記事項 |
|---|
|
ICC
Use at an assay dependent concentration. |
|
IP
Use at an assay dependent concentration. |
|
ICC/IF
Use at an assay dependent concentration. |
|
WB
Use at an assay dependent concentration. |
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
- Entrez Gene: 24115 Mouse
- Entrez Gene: 293735 Rat
- Omim: 607854 Human
- SwissProt: O76090 Human
- SwissProt: O88870 Mouse
- Unigene: 524910 Human
- Unigene: 712676 Human
see all -
別名
- ARB antibody
- BEST 1 antibody
- BEST antibody
see all
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (7)
ab14927 は 7 報の論文で使用されています。
- Zhang H et al. Transplantation of GMP-grade human iPSC-derived retinal pigment epithelial cells in rodent model: the first pre-clinical study for safety and efficacy in China. Ann Transl Med 9:245 (2021). PubMed: 33708872
- McCormick R et al. Optimisation of a Novel Bio-Substrate as a Treatment for Atrophic Age-Related Macular Degeneration. Front Bioeng Biotechnol 8:456 (2020). PubMed: 32500067
- Geng Z et al. Generation of retinal pigmented epithelium from iPSCs derived from the conjunctiva of donors with and without age related macular degeneration. PLoS One 12:e0173575 (2017). PubMed: 28282420
- Lin YH et al. Opening a New Time Window for Treatment of Stroke by Targeting HDAC2. J Neurosci 37:6712-6728 (2017). PubMed: 28592694
- Brennan SC et al. The extracellular calcium-sensing receptor regulates human fetal lung development via CFTR. Sci Rep 6:21975 (2016). PubMed: 26911344
- Guziewicz KE et al. Molecular consequences of BEST1 gene mutations in canine multifocal retinopathy predict functional implications for human bestrophinopathies. Invest Ophthalmol Vis Sci 52:4497-505 (2011). PubMed: 21498618
- Günzel D et al. Claudin-16 affects transcellular Cl- secretion in MDCK cells. J Physiol 587:3777-93 (2009). PubMed: 19528248