Anti-Connexin 43 / GJA1 (phospho S261)抗体(ab62252)

概述

  • 产品名称Anti-Connexin 43 / GJA1 (phospho S261)抗体
    参阅全部 Connexin 43 / GJA1 一抗
  • 描述
    兔多克隆抗体to Connexin 43 / GJA1 (phospho S261)
  • 特异性ab62252 detects endogenous levels of Connexin 43 / GJA1 3 only when phosphorylated at serine 261.
  • 经测试应用适用于: ELISA, IHC-Pmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat
  • 免疫原

    Synthetic phosphopeptide derived from human Connexin 43/ GJA1 around the phosphorylation site of serine 261 (C-G-SP-Q-K).

  • 阳性对照
    • Human brain tissue.

性能

应用

Our Abpromise guarantee covers the use of ab62252 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
ELISA 1/5000.
IHC-P 1/50 - 1/100.

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. May play a critical role in the physiology of hearing by participating in the recycling of potassium to the cochlear endolymph.
  • 组织特异性Expressed in the heart and fetal cochlea.
  • 疾病相关Defects in GJA1 are the cause of autosomal dominant oculodentodigital dysplasia (ODDD) [MIM:164200]; also known as oculodentoosseous dysplasia. ODDD is a highly penetrant syndrome presenting with craniofacial (ocular, nasal, dental) and limb dysmorphisms, spastic paraplegia, and neurodegeneration. Craniofacial anomalies tipically include a thin nose with hypoplastic alae nasi, small anteverted nares, prominent columnella, and microcephaly. Brittle nails and hair abnormalities of hypotrichosis and slow growth are present. Ocular defects include microphthalmia, microcornea, cataracts, glaucoma, and optic atrophy. Syndactyly type 3 and conductive deafness can occur in some cases. Cardiac abnormalities are observed in rare instances.
    Defects in GJA1 are the cause of autosomal recessive oculodentodigital dysplasia (ODDD autosomal recessive) [MIM:257850].
    Defects in GJA1 may be the cause of syndactyly type 3 (SDTY3) [MIM:186100]. Syndactyly is an autosomal dominant trait and is the most common congenital anomaly of the hand or foot. It is marked by persistence of the webbing between adjacent digits, so they are more or less completely attached. In this type there is usually complete and bilateral syndactyly between the fourth and fifth fingers. Usually it is soft tissue syndactyly but occasionally the distal phalanges are fused. The fifth finger is short with absent or rudimentary middle phalanx. The feet are not affected.
    Defects in GJA1 are a cause of hypoplastic left heart syndrome (HLHS) [MIM:241550]. HLHS refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis.
    Defects in GJA1 are a cause of Hallermann-Streiff syndrome (HSS) [MIM:234100]. HSS is a disorder characterized by a typical skull shape (brachycephaly with frontal bossing), hypotrichosis, microphthalmia, cataracts, beaked nose, micrognathia, skin atrophy, dental anomalies and proportionate short stature. Mental retardation is present in a minority of cases.
  • 序列相似性Belongs to the connexin family. Alpha-type (group II) subfamily.
  • 细胞定位Cell membrane. Cell junction > gap junction.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Connexin 43 antibody
    • Connexin-43 antibody
    • Cx 43 antibody
    • Cx43 antibody
    • CXA1_HUMAN antibody
    • DFNB38 antibody
    • Gap junction 43 kDa heart protein antibody
    • Gap junction alpha-1 protein antibody
    • Gap junction protein alpha 1 43kDa (connexin 43) antibody
    • Gap junction protein alpha 1 43kDa antibody
    • Gap junction protein alpha like antibody
    • GJA 1 antibody
    • Gja1 antibody
    • GJAL antibody
    • ODD antibody
    • ODDD antibody
    • ODOD antibody
    • SDTY3 antibody
    see all

Anti-Connexin 43 / GJA1 (phospho S261) antibody 图像

  • ab62252 at 1/50 - 1/100 dilution staining Connexin 43 / GJA1 in Human brain by Immunohistochemistry, Paraffin-embedded tissue, in the absence or presence of the immunising peptide.

Anti-Connexin 43 / GJA1 (phospho S261) antibody (ab62252)参考文献

ab62252 has not yet been referenced specifically in any publications.

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