FITC标记的巴尔得-别德尔综合征相关蛋白2抗体上海沪震实业有限公司

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产品名称: FITC标记的巴尔得-别德尔综合征相关蛋白2抗体
英文名称: Anti-BBS2/FITC
抗体货号: HZ-11506R-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: 2018/8/17 15:45:00
详细资料:  实验方法技术资料
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诚信指数:962点
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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-BBS2/FITC Conjugated antibody

FITC标记的巴尔得-别德尔综合征相关蛋白2抗体

 

产品编号 bs-11506R-FITC
英文名称 Anti-BBS2/FITC
中文名称 FITC标记的巴尔得-别德尔综合征相关蛋白2抗体
别    名 Bardet Biedl syndrome 2; Bardet Biedl syndrome 2 protein; BBS; MGC20703; BBS2_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  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, Rat, Chicken, Dog, Horse, Rabbit, 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 80kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human BBS2
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:
Bardet-Biedl syndrome (BBS) is a pleiotropic genetic disorder characterized by obesity, photoreceptor degeneration, polydactyly, hypogenitalism, renal abnormalities, and developmental delay. Other associated clinical findings in BBS patients include diabetes, hypertension, and congenital heart defects. BBS is a heterogeneous disorder that maps to eight genetic loci and encodes eight proteins, BBS1-BBS8. Five BBS genes encode basal body or cilia proteins, suggesting that BBS is a ciliary dysfunction disorder. BBS2 is a 721-amino acid protein that is evolutionarily conserved and is expressed in a broad range of tissues including: brain, kidney, adrenal gland, and thyroid gland. Loss of BBS2 may be involved in defects in social interactions as well as infertility. BBS2 retinopathy involves normal retina development followed by apoptotic death of photoreceptors, the primary ciliated cells of the retina.

Function:
Defects in BBS2 are the cause of Bardet-Biedl syndrome type 2. Bardet-Biedl syndrome is a genetically heterogeneous, autosomal recessive disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. 

Subunit:
Part of BBSome complex, that contains BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 and BBIP10. The BBSome complex binds to PCM1 and tubulin. Interacts (via C-terminus) with BBS7. Interacts (via coiled coil domain) with MKKS. Interacts with CCDC28B.

Subcellular Location:
Cytoplasmic 

Tissue Specificity:
Widely expressed.

DISEASE:
Defects in BBS2 are the cause of Bardet-Biedl syndrome type 2 (BBS2) [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. 

Database links:
UniProtKB/Swiss-Prot: Q9BXC9.1

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

BARDET Biedl综合征(Baldt Biedl综合征)是一种多效性遗传性疾病,其特征是肥胖、感光细胞退化、多指畸形、肾功能减退、肾功能异常和发育迟缓。BBS患者的其他相关临床表现包括糖尿病、高血压和先天性心脏缺陷。BBS是一种异质性障碍,映射到八个基因位点,编码八个蛋白BBS1-BBS8。五个BBS基因编码基体或纤毛蛋白,提示BBS是睫状体功能障碍。BBS2是一种进化保守的721氨基酸蛋白,广泛表达于脑、肾、肾上腺、甲状腺等组织中。BBS2的丢失可能参与了社会交往和不育的缺陷。BBS2视网膜病变包括视网膜正常发育,视网膜感光细胞的凋亡死亡,主要是纤毛细胞。

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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