| 產(chǎn)品編號 | bs-1715R-BF555 |
| 英文名稱 | Rabbit Anti-Connexin 26/BF555 Conjugated antibody |
| 中文名稱 | BF555標(biāo)記的間隙連接蛋白26/GJB2抗體 |
| 別 名 | cx26; DFNA3; DFNB1; HID; KID; NSRD1; Connexin26; Connexin-26; GJB2; PPK; CXB2_HUMAN. |
| 規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, ) |
| 產(chǎn)品應(yīng)用 | IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 26kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Connexin-26 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol |
| 保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 產(chǎn)品介紹 |
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 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 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. Database links: Entrez Gene: 2706 Human Entrez Gene: 14619 Mouse Omim: 121011 Human SwissProt: P29033 Human SwissProt: Q00977 Mouse 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. 間隙連接(Gap junction, GJ)是普遍存在于相鄰細(xì)胞間的細(xì)胞連接方式，是相鄰細(xì)胞膜上的一種膜蛋白通道結(jié)構(gòu)。間隙連接允許鄰近細(xì)胞之間的離子，核苷酸和小的調(diào)節(jié)分子以及藥物或它們的代謝物進(jìn)行轉(zhuǎn)運，細(xì)胞通過它所介導(dǎo)的細(xì)胞間連接通訊,進(jìn)行細(xì)胞間信息和能量的傳遞，調(diào)控細(xì)胞的生長、分化和內(nèi)環(huán)境的穩(wěn)定，對維持機(jī)體的功能發(fā)揮重要作用. 間隙連接蛋白26目前主要用于腫瘤方面的研究。 |
