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BEST1抗体BEST1一抗抗体Vitelliform ma

cular dystrophy protein 2抗体BEST1 Antibody抗体
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  • ¥1100 - 1980
  • CUSABIO已认证
  • CN
  • CSB-PA269030
  • 2025年07月11日
  • ELISA,WB,IHC;ELISA:1:1000-1:2000,WB:1:200-1:1000,IHC:1:25-1:100
  • Rabbit
  • Human,Mouse
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    • 详细信息
    • 文献和实验
    • 技术资料
    • 免疫原

      Synthetic peptide of Human BEST1

    • 亚型

      IgG

    • 形态

      Liquid

    • 保存条件

      Upon receipt, store at -20℃ or -80℃. Avoid repeated freeze.

    • 克隆性

      /

    • 标记物

      Non-conjugated

    • 适应物种

      Human,Mouse

    • 保质期

      6个月

    • 抗原来源

      Homo sapiens (Human)

    • 目录编号

      O76090

    • 级别

    • 库存

      200

    • 供应商

      武汉华美生物工程有限公司

    • 宿主

      Rabbit

    • 应用范围

      ELISA,WB,IHC;ELISA:1:1000-1:2000,WB:1:200-1:1000,IHC:1:25-1:100

    • 浓度

      >95%,Antigen affinity purification

    • 靶点

      BEST1

    • 抗体英文名

      BEST1 Antibody

    • 抗体名

      BEST1;VMD2;Bestrophin-1;TU15B;Vitelliform macular dystrophy protein 2

    • 规格

      100μl/50μl

    规格:100μl产品价格:¥1980.0
    规格:50μl产品价格:¥1100.0

    保存缓冲液

    -20℃, pH7.4 PBS, 0.05% NaN3, 40% Glycerol

    功能

    This gene encodes a member of the bestrophin gene family. This small gene family is characterized by proteins with a highly conserved N-terminus with four to six transmembrane domains. Bestrophins may form chloride ion channels or may regulate voltage-gated L-type calcium-ion channels. Bestrophins are generally believed to form calcium-activated chloride-ion channels in epithelial cells but they have also been shown to be highly permeable to bicarbonate ion transport in retinal tissue. Mutations in this gene are responsible for juvenile-onset vitelliform macular dystrophy (VMD2), also known as Best macular dystrophy, in addition to adult-onset vitelliform macular dystrophy (AVMD) and other retinopathies. Alternative splicing results in multiple variants encoding distinct isoforms.

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    相关实验
    • Monitoring Duchenne Muscular Dystrophy Gene Therapy with Epitope-Specific Monoclonal Antibodies

      Several molecular approaches to Duchenne muscular dystrophy (DMD) therapy are at or near the point of clinical trial and usually involve attempts to replace the missing dystrophin protein. Although improved muscle function is the ultimate

    • ABC Transporters in Ophthalmic Disease

      ABC transporters have been implicated in a variety of human diseases. The ABCR gene and its protein have been linked to Stargardt’s disease, fundus flavimaculatus, cone–rod dystrophy, retinitis pigmentosa, and age-related macular degeneration

    • Retinal Tissue

      and are thereby expressed exclusively in photoreceptor cells. However, when altered, genes for RPE65, RPE-retinal G protein-coupled receptor (RGR), cellular retinaldehyde-binding protein (CRALBP), and Best macular dystrophy (VMD2), which are expressed in the RPE

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    文献支持
    BEST1抗体BEST1一抗抗体Vitelliform macular dystrophy protein 2抗体BEST1 Antibody抗体
    ¥1100 - 1980