间隙连接蛋白26/GJB2抗体
规格：1mg/1ml
英文名： Connexin 26
别名： cx26; DFNA3; DFNB1; HID; KID; NSRD1; Connexin26; Connexin-26; GJB2; PPK; CXB2_HUMAN.
分子量： 26kDa
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, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞膜
间隙连接蛋白26/GJB2抗体产品介绍：background: Gap junction channels consist of connexin protein subunits, which are encoded by a multigene family. GJBs (gap junction proteins or connexins) play crucial functional roles associated with these channels. Immunohistochemical staining of human cochlear cells demonstrated high levels of GJB2 expression. Mutations in GJB2 are associated with genetically derived hearing impairments, including autosomal recessive nonsyndromic deafness. 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. May play a critical role in the physiology of hearing by participating in the recycling of potassium to the cochlear endolymph. Subcellular Location: Cell membrane. Cell junction, gap junction. Tissue Specificity: Expressed in the heart and fetal cochlea. Post-translational modifications: Belongs to the connexin间隙连接蛋白26/GJB2抗体 family. Beta-type (group I) subfamily. DISEASE: 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间隙连接蛋白26/GJB2抗体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]. Similarity: Belongs to the connexin family. Alpha-type (group II) subfamily. Gene ID: 2706 Database links: Entrez Gene: 2706 Human Entrez Gene: 14619 Mouse Entrez Gene: 394266 Rat Omim: 121011 Human SwissProt: P29033 Human SwissProt: Q00977 Mouse SwissProt: P21994 Rat Unigene: 524894 Human Unigene: 714494 Human Unigene: 390683 Mouse Unigene: 219269 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(Gap junction, GJ)是普遍存在于相邻细胞间的细胞连间隙连接蛋白26/GJB2抗体接方式，是相邻细胞膜上的一种膜蛋白通道结构。间隙连接允许邻近细胞之间的离子，核苷酸和小的调节分子以及**或它们的代谢物进行转运，细胞通过它所介导的细胞间连接通讯,进行细胞间信息和能量的传递，调控细胞的生长、分化和内环境的稳定，对维持机体的功能发挥重要作用. 间隙连接蛋白26目前主要用于肿瘤方面的研究。
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：肿瘤 细胞生物 神经生物学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid