概述

  • 产品名称
    Anti-Bestrophin抗体
    参阅全部 Bestrophin 一抗
  • 描述
    兔多克隆抗体to Bestrophin
  • 经测试应用
    适用于: WB, ICC/IFmore details
  • 种属反应性
    与反应: Rat, Human
    预测可用于: Cynomolgus monkey
  • 免疫原

    Synthetic peptide corresponding to Human Bestrophin aa 445-464.
    Sequence:

    WKLKAVDAFKSAPLYQRPGY

  • 阳性对照
    • Conditioned medium cultured RPE cells (the basal expression of Bestrophin is low in cultured cells).

性能

应用

Our Abpromise guarantee covers the use of ab14928 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
ICC/IF
  • 应用说明
    ICC/IF: Use at an assay dependent dilution.
    WB: 1/500. Predicted molecular weight: 68 kDa.

    Not tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 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
    • 数据库链接
    • 别名
      • ARB antibody
      • BEST 1 antibody
      • BEST antibody
      • Best disease antibody
      • Best macular dystrophy antibody
      • BEST1 antibody
      • BEST1_HUMAN antibody
      • Best1V1Delta2 antibody
      • Bestrophin 1 antibody
      • Bestrophin-1 antibody
      • Bestrophin1 antibody
      • BMD antibody
      • mBest1 antibody
      • RP50 antibody
      • TU15B antibody
      • Vitelliform macular dystrophy 2 antibody
      • Vitelliform macular dystrophy antibody
      • Vitelliform macular dystrophy protein 2 antibody
      • VMD 2 antibody
      • VMD2 antibody
      see all

    图片

    • Immunofluorescence analysis of hESC-RPE monolayers, staining Bestrophin with ab14928.

      Cells were fixed with 4% paraformaldehyde, permeabilized with 0.1% Triton X-100 and blocked with 3% BSA for 1 hour. Cells were incubated with primary antibody (1/500) for 1 hour at room temperature. An AlexaFluor®568-conjugated goat anti-rabbit IgG (1/800) was used as the secondary antibody.

    文献

    This product has been referenced in:
    • Subrizi A  et al. Generation of hESC-derived retinal pigment epithelium on biopolymer coated polyimide membranes. Biomaterials 33:8047-54 (2012). ICC/IF ; Human . Read more (PubMed: 22892561) »
    • 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). Read more (PubMed: 21498618) »

    See all 2 Publications for this product

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    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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