FITC标记的巴尔得-别德尔综合征相关蛋白12抗体上海沪震实业有限公司

性能参数

产品名称: FITC标记的巴尔得-别德尔综合征相关蛋白12抗体
英文名称: Anti-BBS12/FITC
抗体货号: HZ-11505R-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: 2018/7/21 11:16:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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上海沪震实业有限公司
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产品详细描述

 Rabbit Anti-BBS12/FITC Conjugated antibody

FITC标记的巴尔得-别德尔综合征相关蛋白12抗体

 

产品编号 bs-11505R-FITC
英文名称 Anti-BBS12/FITC
中文名称 FITC标记的巴尔得-别德尔综合征相关蛋白12抗体
别    名 Bardet Biedl syndrome 12 protein; Bardet-Biedl syndrome 12 (human); Bardet-Biedl syndrome 12 protein homolog; BBS12 gene; C4orf24; FLJ35630; FLJ41559; Gm1805; Gm407; Gm721; RP23-137F6.2;BBS12_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  细胞生物  神经生物学  内分泌病  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Cow, Horse, Rabbit, 
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 79kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human BBS12
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:
The protein encoded by this gene is part of a complex that is involved in membrane trafficking. The encoded protein is a molecular chaperone that aids in protein folding upon ATP hydrolysis. This protein also plays a role in adipocyte differentiation. Defects in this gene are a cause of Bardet-Biedl syndrome type 12. Two transcript variants encoding the same protein have been found for this gene.

Function:
Probable molecular chaperone. Assists the folding of proteins upon ATP hydrolysis. As part of the BBS/CCT complex may play a role in the assembly of BBSome, a complex involved in ciliogenesis regulating transports vesicles to the cilia. Involved in adipogenic differentiation.

Subunit:
Component of the BBS/CCT complex composed at least of MKKS, BBS10, BBS12, TCP1, CCT2, CCT3, CCT4, CCT5 AND CCT8.

Subcellular Location:
Cell projection, cilium. Note: Located within the basal body of the primary cilium of differentiating preadipocytes.

DISEASE:
Defects in BBS12 are the cause of Bardet-Biedl syndrome type 12 (BBS12) [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. BBS12 seems to be rarely involved in oligogenic inheritance.

Similarity:
Belongs to the TCP-1 chaperonin family. BBS12 subfamily.

Database links:
UniProtKB/Swiss-Prot: Q6ZW61.2

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

由该基因编码的蛋白质是参与膜运输的复合物的一部分。编码的蛋白质是分子伴侣,在ATP水解时有助于蛋白质折叠。这种蛋白质也在脂肪细胞分化中起作用。该基因的缺陷是BARDET BieDL综合征12型的一个原因。已经发现了两个编码相同蛋白的转录变体。

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。

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