FITC标记的梅克尔-格鲁伯综合征相关蛋白抗体上海沪震实业有限公司

性能参数

产品名称: FITC标记的梅克尔-格鲁伯综合征相关蛋白抗体
英文名称: Anti-MKS1/FITC
抗体货号: HZ-11513R-FITC
产品规格: 100ul
级    别: 分析纯, , 分析纯,
产品产地: 中国/上海
品牌商标: HZbscience
价    格: 2980元
抗原: Rabbit
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: 
FITCconjugate:FITC
应用范围: ICC=1:50-200 IF=1:50-200
更新时间: 2018/6/14 8:59:00
详细资料:  实验方法技术资料
浏览人数:7
诚信指数:754点
了解更多:进入公司展台
使用范围:仅限科研使用,不能应用于临床
点此求购
在线QQ: 我是2043711056,请问我有什么可以帮到您呢?
生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

供应商联系卡

上海沪震实业有限公司
地址:
上海市杨浦区密云路1018号复旦科技园808室
邮编:
200612
电话:
021-60345367
传真:
021-31320307
联系人:
陈先生
所在区域:
上海·中国-上海
邮件:
公司展台:
在线QQ:
我是2043711056,请问我有什么可以帮到您呢?
扫一扫,关注我们

产品详细描述

 Rabbit Anti-MKS1/FITC Conjugated antibody

FITC标记的梅克尔-格鲁伯综合征相关蛋白抗体

 

产品编号 bs-11513R-FITC
英文名称 Anti-MKS1/FITC
中文名称 FITC标记的梅克尔-格鲁伯综合征相关蛋白抗体
别    名 MES; B8d3; BBS13; Dysencephalia splanchnocystica; FABB proteome like protein; FLJ20345; Gruber syndrome; Meckel gruber syndrome; Meckel gruber syndrome type 1; Meckel syndrome; Meckel syndrome type 1; Meckel syndrome type 1 protein; Meckel syndrome type 1 protein homolog; MKS 1; MKS; MKS1; POC12; POC12 centriolar protein homolog.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  结合蛋白  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, 
产品应用 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 64kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MKS1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 background:
MKS1 is involved in centrosome migration to the apical cell surface during early ciliogenesis. It is required for ciliary structure and function, including a role in regulating length and appropriate number through modulating centrosome duplication. It is also required for cell branching morphology.

Function:
Component of the tectonic-like complex, a complex localized at the transition zone of primary cilia and acting as a barrier that prevents diffusion of transmembrane proteins between the cilia and plasma membranes. Involved in centrosome migration to the apical cell surface during early ciliogenesis. Required for ciliary structure and function, including a role in regulating length and appropriate number through modulating centrosome duplication. Required for cell branching morphology. 

Subunit:
Part of the tectonic-like complex (also named B9 complex) (By similarity). Interacts with TCTN3 and AHI1 (By similarity). Interacts with FLNA.

Subcellular Location:
Cytoplasm, cytoskeleton, cilium basal body. Cytoplasm, cytoskeleton, centrosome. Note: Localizes at the transition zone, a region between the basal body and the ciliary axoneme 

DISEASE:
Defects in MKS1 are the cause of Meckel syndrome type 1 (MKS1) [MIM:249000]. MKS1 is an autosomal recessive disorder characterized by a combination of renal cysts and variably associated features including developmental anomalies of the central nervous system (typically encephalocele), hepatic ductal dysplasia and cysts, and polydactyly. 
Defects in MKS1 are the cause of Bardet-Biedl syndrome type 13 (BBS13) [MIM:209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous, autosomal recessive disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. A relatively high incidence of BBS is found in the mixed Arab populations of Kuwait and in Bedouin tribes throughout the Middle East, most likely due to the high rate of consaguinity in these populations and a founder effect. 

Similarity:
Contains 1 B9 domain. 

Database links:
UniProtKB/Swiss-Prot: Q9NXB0.2

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications
   

MKS1参与了纤毛发生过程中心体向顶端细胞表面的迁移。纤毛结构和功能是必需的,包括调节中心体复制调节长度和适当数量的作用。这也是细胞分枝形态所必需的。

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

查看FITC标记的梅克尔-格鲁伯综合征相关蛋白抗体产品的用户还对以下产品感兴趣

暂无

ADVERTISEMENT

找不到您所需的产品,发布求购试试?

标题:*
描述:
姓名:*
Email:*
单位:*
电话:*
地址:*
邮编:
资料: 需要 不需要
报价: 需要 不需要