Anti-Bestrophin/BEST1 抗体 (ab14929)
Key features and details
- Rabbit polyclonal to Bestrophin/BEST1
- Suitable for: IHC-P
- Reacts with: 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: IHC-Pmore details -
種交差性
交差種: Human -
免疫原
Synthetic peptide corresponding to Human Bestrophin/BEST1 aa 550-650 (C terminal).
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. -
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ab14929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 |
|---|---|---|
| IHC-P |
Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
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| 特記事項 |
|---|
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IHC-P
Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. |
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
- 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 (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Bestrophin/BEST1 antibody (ab14929)IHC image of ab14929 staining in human normal hippocampus formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab14929, 5µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.
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)
ab14929 は 7 報の論文で使用されています。
- Gu Q et al. Accreditation of Biosafe Clinical-Grade Human Embryonic Stem Cells According to Chinese Regulations. Stem Cell Reports 9:366-380 (2017). PubMed: 28506532
- Wu W et al. Features specific to retinal pigment epithelium cells derived from three-dimensional human embryonic stem cell cultures - a new donor for cell therapy. Oncotarget 7:22819-33 (2016). PubMed: 27009841
- Sohn EH et al. Allogenic iPSC-derived RPE cell transplants induce immune response in pigs: a pilot study. Sci Rep 5:11791 (2015). IHC-Fr . PubMed: 26138532
- Tucker BA et al. Patient-specific iPSC-derived photoreceptor precursor cells as a means to investigate retinitis pigmentosa. Elife 2:e00824 (2013). IHC . PubMed: 23991284
- 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
- Chen M et al. Characterization of a spontaneous mouse retinal pigment epithelial cell line B6-RPE07. Invest Ophthalmol Vis Sci 49:3699-706 (2008). WB ; Mouse . PubMed: 18421091
- Mullins RF et al. Differential macular and peripheral expression of bestrophin in human eyes and its implication for best disease. Invest Ophthalmol Vis Sci 48:3372-80 (2007). IHC ; Human . PubMed: 17591911