| 產(chǎn)品編號 | bs-23115R |
| 英文名稱 | connexin 30 Rabbit pAb |
| 中文名稱 | 間隙連接蛋白30/GJB6抗體 |
| 別 名 | Connexin 30; Connexin-30; Cx30; CXB6_HUMAN; DFNA3; ectodermal dysplasia 2, hidrotic(Clouston syndrome); ED2; EDH; Gap junction beta 6 protein; Gap junction beta-6 protein; gap junction protein, beta 6(connexin 30); gap junction protein, beta 6; GJB6; HED; NSRD1. |
| 研究領(lǐng)域 | 細(xì)胞生物 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo) 細(xì)胞粘附分子 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse,Rat (predicted: Human,Rabbit,Sheep,Cow,Horse) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 29 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human connexin-30: 201-261/261 <Cytoplasmic> |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
The connexin family of proteins form hexameric complexes called connexons that facilitate movement of low molecular weight proteins between cells via gap junctions. Connexin proteins share a common topology of four transmembrane alpha-helical domains, two extracellular loops, a cytoplasmic loop and cytoplasmic N- and C-termini. Many of the key functional differences between connexins arise from specific amino-acid substitutions in the most highly conserved domains: the transmembrane and extracellular regions. Connexin 30, also known as GJB6 (Gap junction beta 6), ED2, EDH, HED or DFNA3, is a 261 amino acid multi-pass membrane protein that localizes to the cell junction and belongs to the connexin family. Functioning as a hexamer with other connexin proteins, connexin 30 facilitates the diffusion of low molecular weight materials from one cell to another. Defects in the gene encoding connexin 30 are the cause of ectodermal dysplasia type 2 (ED2) and non-syndromatic sensorineural deafness autosomal dominant type 3 (DFNA3), the former of which is characterized by abnormal development of ectodermal structures (such as skin and nails). 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 GJB6 are the cause of ectodermal dysplasia type 2 (ED2) [MIM:129500]; also known as Clouston syndrome. Ectodermal dysplasia defines a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. ED2 is an autosomal dominant condition characterized by atrichosis, nail hypoplasia and deformities, hyperpigmentation of the skin, normal teeth, normal sweat and sebaceous gland function. Palmoplantar hyperkeratosis is a frequent features. Hearing impairment has been detected in few cases of ED2. Defects in GJB6 are the cause of deafness autosomal recessive type 1B (DFNB1B) [MIM:612645]. DFNB1B 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. Defects in GJB6 are the cause of deafness autosomal dominant type 3B (DFNA3B) [MIM:612643]. Similarity: Belongs to the connexin family. Beta-type (group I) subfamily. SWISS: O95452 Gene ID: 10804 Database links: Entrez Gene: 10804 Human Entrez Gene: 14623 Mouse SwissProt: O95452 Human SwissProt: P70689 Mouse |
| 產(chǎn)品圖片 |
Sample:
Lane 1: Mouse Cerebrum tissue lysates
Lane 2: Mouse Skin tissue lysates
Lane 3: Mouse Lung tissue lysates
Lane 4: Rat Cerebrum tissue lysates
Lane 5: Rat Lung tissue lysates
Primary: Anti-connexin 30 (bs-23115R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 29 kDa
Observed band size: 27 kDa
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