Anti-GJB6抗体(ab97428)

概述

  • 产品名称Anti-GJB6抗体
    参阅全部 GJB6 一抗
  • 描述
    兔多克隆抗体to GJB6
  • 经测试应用适用于: ICC/IF, WBmore details
  • 种属反应性
    与反应: Mouse
    预测可用于: Rat
  • 免疫原

    Synthetic peptide conjugated to KLH derived from within residues 200 to the C-terminus of Human GJB6.

    (Peptide available as ab109645.)

  • 阳性对照
    • This antibody gave a positive signal in Mouse Retina tissue lysate.

性能

应用

Our Abpromise guarantee covers the use of ab97428 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
ICC/IF Use a concentration of 5 µg/ml.
WB Use a concentration of 1 µg/ml. Detects a band of approximately 28 kDa (predicted molecular weight: 30 kDa).

靶标

  • 功能One gap junction consists of a cluster of closely packed pairs of transmembrane channels, the connexons, through which materials of low MW diffuse from one cell to a neighboring cell.
  • 疾病相关Defects in GJB6 are the cause of ectodermal dysplasia type 2 (ED2) [MIM:129500]; also known as Clouston syndrome. Ectodermal dysplasia defines a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. ED2 is an autosomal dominant condition characterized by atrichosis, nail hypoplasia and deformities, hyperpigmentation of the skin, normal teeth, normal sweat and sebaceous gland function. Palmoplantar hyperkeratosis is a frequent features. Hearing impairment has been detected in few cases of ED2.
    Defects in GJB6 are the cause of deafness autosomal recessive type 1B (DFNB1B) [MIM:612645]. DFNB1B is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information.
    Defects in GJB6 are the cause of deafness autosomal dominant type 3B (DFNA3B) [MIM:612643].
  • 序列相似性Belongs to the connexin family. Beta-type (group I) subfamily.
  • 细胞定位Cell membrane. Cell junction > gap junction.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Connexin 30 antibody
    • Connexin-30 antibody
    • Cx30 antibody
    • CXB6_HUMAN antibody
    • DFNA3 antibody
    • DFNA3B antibody
    • DFNB1B antibody
    • ECTD2 antibody
    • ectodermal dysplasia 2, hidrotic (Clouston syndrome) antibody
    • ED2 antibody
    • EDH antibody
    • Gap junction beta 6 protein antibody
    • Gap junction beta-6 protein antibody
    • gap junction protein, beta 6 (connexin 30) antibody
    • gap junction protein, beta 6 antibody
    • Gap junction protein, beta 6, 30kDa antibody
    • GJB6 antibody
    • HED antibody
    • HED2 antibody
    • NSRD1 antibody
    see all

Anti-GJB6 antibody 图像

  • Anti-GJB6 antibody (ab97428) at 1 µg/ml + Mouse Retina Tissue Lysate at 10 µg

    Secondary
    Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 30 kDa
    Observed band size : 28 kDa (why is the actual band size different from the predicted?)


    Exposure time : 8 minutes
  • ICC/IF image of ab97428 stained PC12 cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody ab97428 at 5µg/ml overnight at +4°C. The secondary antibody (green) was DyLight® 488 goat anti- rabbit (ab96899) IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.

Anti-GJB6 antibody (ab97428)参考文献

ab97428 has not yet been referenced specifically in any publications.

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