间隙连接蛋白31抗体
规格:1mg/1ml
英文名: GJB3
别名: Connexin 31; Connexin-31; Connexin31; CX 31; Cx31; CXB3_HUMAN; DFNA 2; DFNA2; DFNA2B; EKV; FLJ22486; Gap junction beta 3 protein; Gap junction beta-3 protein; Gap junction protein beta 3 31kDa; Gap ju
分子量: 31kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:Polyclonal
亚型:IgG
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human Connexin
交叉反应:Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig,
细胞定位:细胞膜
间隙连接蛋白31抗体产品介绍:background: This gene is a member of the connexin gene family. The encoded protein is a component of gap junctions, which are composed of arrays of intercellular channels that provide a route for the diffusion of low molecular weight materials from cell to cell. Mutations in this gene can cause non-syndromic deafness or erythrokeratodermia variabilis, a skin disorder. Alternative splicing results in multiple transcript variants encoding the same protein. [provided by RefSeq, Jul 2008]. Function: 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. Subunit: A connexon is composed of a hexamer of connexins. Interacts with CNST. Subcellular Location: Cell membrane; Multi-pass membrane protein. Cell junction, gap junction. DISEASE: Defects in GJB3 are a cause of erythrokeratodermia variabilis (EKV) [MIM:133200].间隙连接蛋白31抗体 EKV is a genodermatosis characterized by the appearance of two independent skin lesions: transient figurate erythematous patches and hyperkeratosis that is usually localized but occasionally occurs in its generalized form. Clinical presentation varies significantly within a family and from one family to another. Palmoplantar keratoderma is present in around 50% of cases. Defects in GJB3 are the cause of deafness autosomal dominant type 2B (DFNA2B) [MIM:612644]. DFNA2 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. Similarity: Belongs to间隙连接蛋白31抗体 the connexin family. Beta-type (group I) subfamily. Database links: Entrez Gene: 2707 Human Entrez Gene: 14620 Mouse Entrez Gene: 29585 Rat Omim: 603324 Human SwissProt: O75712 Human SwissProt: P28231 Mouse SwissProt: P25305 Rat Unigene: 522561 Human Unigene: 90003 Mouse Unigene: 162823 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
间隙连接蛋白31抗体产品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域:**学 神经生物学 信号转导 细胞膜受体
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid