概述

  • 产品名称
  • 描述
    兔多克隆抗体to GJB6
  • 经测试应用
    适用于: WB, IHC-P, ELISAmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide corresponding to Human GJB6 aa 87-117 (internal sequence) conjugated to keyhole limpet haemocyanin.
    Sequence:

    PALL VAMHVAYYRH ETTRKFR


    Database link: O95452

  • 阳性对照
    • Human breast carcinoma tissue

性能

  • 形式
    Liquid
  • 存放说明
    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term.
  • 存储溶液
    Preservative: 0.09% Sodium Azide
    Constituents: PBS
  • Concentration information loading...
  • 纯度
    Protein G purified
  • 纯化说明
    This antibody is purified through a protein G column and eluted out with both high and low pH buffers and neutralized immediately after elution then followed by dialysis against PBS.
  • 克隆
    多克隆
  • 同种型
    IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab59927 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 1/1000. Predicted molecular weight: 30 kDa.
IHC-P 1/50 - 1/100.
ELISA 1/1000.

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

图片

  • Formalin fixed and paraffin embedded human breast carcinoma tissue labelled with ab59927 at 1/50 dilution, followed by peroxidase-conjugation to the secondary antibody and AEC staining.
  • All lanes : Anti-GJB6 antibody (ab59927) at 500 µg/ml

    Lane 1 : Non-transfected 293 cell lysates
    Lane 2 : GJB6 gene transfected 293 cells lysates

    Lysates/proteins at 2 µg per lane.


    Predicted band size : 30 kDa
  • ab59927 at 1/100 dilution staining GJB6 in human prostate carcinoma tissue section by Immunohistochemistry (Formalin/ PFA fixed paraffin-embedded sections). A peroxidase conjugated secondary antibody was used followed by DAB staining.

文献

ab59927 has not yet been referenced specifically in any publications.

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