Anti-Connexin 32 / GJB1抗体(ab11368)

概述

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 or -80°C. Avoid freeze / thaw cycle.
  • 存储溶液
    Preservative: 15mM Sodium Azide
    Constituents: 1% BSA, 0.01M PBS (pH7.4)
  • Concentration information loading...
  • 纯度
    Immunogen affinity purified
  • 克隆
    多克隆
  • 同种型
    IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab11368 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 1/600. Detects a band of approximately 27 kDa.
IHC-Fr 1/400.

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 GJB1 are the cause of Charcot-Marie-Tooth disease X-linked type 1 (CMTX1) [MIM:302800]; also designated CMT-X. CMTX1 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy. CMTX1 has both demyelinating and axonal features. Central nervous system involvement may occur.
    Defects in GJB1 may contribute to the phenotype of Dejerine-Sottas syndrome (DSS) [MIM:145900]; also known as Dejerine-Sottas neuropathy (DSN) or hereditary motor and sensory neuropathy III (HMSN3). DSS is a severe degenerating neuropathy of the demyelinating Charcot-Marie-Tooth disease category, with onset by age 2 years. DSS is characterized by motor and sensory neuropathy with very slow nerve conduction velocities, increased cerebrospinal fluid protein concentrations, hypertrophic nerve changes, delayed age of walking as well as areflexia. There are both autosomal dominant and autosomal recessive forms of Dejerine-Sottas syndrome.
  • 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
    • Charcot Marie Tooth neuropathy X linked antibody
    • CMTX 1 antibody
    • CMTX antibody
    • CMTX1 antibody
    • Connexin-32 antibody
    • Connexin32 antibody
    • CX 32 antibody
    • Cx32 antibody
    • CXB1_HUMAN antibody
    • GAP junction 28 kDa liver protein antibody
    • Gap junction beta 1 protein antibody
    • Gap junction beta-1 protein antibody
    • Gap junction protein beta 1 32kD antibody
    • Gap junction protein beta 1 antibody
    • GJB 1 antibody
    • GJB1 antibody
    see all

文献

ab11368 has not yet been referenced specifically in any publications.

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