无码影视中文高清_亚洲天堂光棍影院_欧美激情欧美激情在线五月_国产精品日韩免费观看_一级黄色毛片成人影院_六月综合激情_亚洲av无码区国产乱码粉嫩_无遮挡免费视频_97操射操射人人色_可以免费观看的黄色完整版网站视频

掃碼關(guān)注公眾號           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務(wù)
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  人才招聘  關(guān)于我們  聯(lián)系我們
欢乐颂第一季,盗墓笔记第二季
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-connexin 30/Cy5.5 Conjugated antibody (bs-23115R-Cy5.5)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-23115R-Cy5.5
英文名稱1 Rabbit Anti-connexin 30/Cy5.5 Conjugated antibody
中文名稱 Cy5.5標記的間隙連接蛋白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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 細胞生物  神經(jīng)生物學  信號轉(zhuǎn)導  細胞粘附分子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,  (predicted: Human, Rat, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 29kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human connexin
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:
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.

Database links:

Entrez Gene: 10804 Human

Entrez Gene: 14623 Mouse

SwissProt: O95452 Human

SwissProt: P70689 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權(quán)所有 2004-2026 xiaowangchong.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號