Anti-Connexin 43 / GJA1抗体(ab62689)

概述

• 产品名称Anti-Connexin 43 / GJA1抗体
  参阅全部 Connexin 43 / GJA1 一抗
• 描述
  兔多克隆抗体to Connexin 43 / GJA1
• 特异性Detects endogenous levels of total Connexin 43 / GJA1 protein.
• 经测试应用适用于: ELISA, ICC/IF, IHC-Pmore details
• 种属反应性
  与反应: Human
  不与反应: Mouse
• 免疫原

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

• 阳性对照
  • Brain. HeLa cells.

性能

• 形式Liquid
• 存放说明Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
• 存储溶液Preservative: 0.02% Sodium Azide
  Constituents: 50% Glycerol, PBS (without Mg²⁺ and Ca²⁺), 150mM Sodium chloride, pH 7.4
• Concentration information loading...
• 纯度Immunogen affinity purified
• 克隆多克隆
• 同种型IgG
• 研究领域
  • Signal Transduction
  • Cytoskeleton / ECM
  • Cell Adhesion
  • Gap Junctions

  • Cardiovascular
  • Atherosclerosis
  • Vascular Inflammation
  • Leukocyte recruitment
  • Cell junction molecules

  • Cardiovascular
  • Heart
  • Cardiac arrhythmias

相关产品

• Compatible Secondaries
  • Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) (ab150077)
  • Goat Anti-Rabbit IgG H&L (HRP) (ab205718)

应用

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.

• Target information above from: UniProt accession P17302 The UniProt Consortium
  The Universal Protein Resource (UniProt) in 2010
  Nucleic Acids Res. 38:D142-D148 (2010) .

  Information by UniProt
• 数据库链接
  • Entrez Gene: 2697 Human
  • Omim: 121014 Human
  • SwissProt: P17302 Human
  • Unigene: 74471 Human
• 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