FITC标记的巴尔得-别德尔综合征相关蛋白9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9抗体
英文名称: Anti-BBS9/FITC
抗体货号: HZ-11511R-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: 2018/7/28 14:06:00
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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产品详细描述

 Rabbit Anti-BBS9/FITC Conjugated antibody

FITC标记的巴尔得-别德尔综合征相关蛋白9抗体

 

产品编号 bs-11511R-FITC
英文名称 Anti-BBS9/FITC
中文名称 FITC标记的巴尔得-别德尔综合征相关蛋白9抗体
别    名 B1 antibody; Bardet Biedl syndrome 9; Bardet-Biedl syndrome 9 protein; bbs9; C18 antibody D1 antibody MGC118917; 1 gene protein; Protein PTHB1; PTH-responsive osteosarcoma B1 protein; PTHB1; PTHB1_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  信号转导  生长因子和***  内分泌病  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, 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 99kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human BBS9
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:
BBS9 is an 887 amino acid protein that localizes to both the cytoplasm and the centrosome and exists as six alternatively spliced isoforms. Expressed in a wide variety of tissues, including liver, lung, heart, brain and skeletal muscle, BBS9 functions as a component of the multi-protein BBSome complex which is required for ciliogenesis and is regulated by GDP/GTP exchange factors. Defects in the gene encoding BBS9 are associated with the pathogenesis of Bardet-Biedl syndrome type 9 (BBS9), an autosomal recessive disorder that is characterized by severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Additionally, chromosomal aberrations involving the BBS9 gene may play a role in the formation of Wilms tumor 5 (WT5).

Function:
The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane and binds to RAB3IP/Rabin8, the guanosyl exchange factor (GEF) for Rab8 and then the Rab8-GTP localizes to the cilium and promotes docking and fusion of carrier vesicles to the base of the ciliary membrane.

Subunit:
Part of BBSome complex, that contains BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 and BBIP10. The BBSome complex binds to PCM1 and tubulin. 

Subcellular Location:
Cytoplasm, cytoskeleton, centrosome. Cell projection, cilium membrane. Cytoplasm. Note=Localizes to nonmembranous centriolar satellites in the cytoplasm.

Tissue Specificity:
Widely expressed. Expressed in adult heart, skeletal muscle, lung, liver, kidney, placenta and brain, and in fetal kidney, lung, liver and brain.

DISEASE:
Defects in BBS9 are a cause of Bardet-Biedl syndrome type 9 (BBS9) [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.

Database links:

Entrez Gene: 27241 Human

Entrez Gene: 319845 Mouse

Entrez Gene: 315484 Rat

Omim: 607968 Human

SwissProt: Q3SYG4 Human

SwissProt: Q811G0 Mouse

Unigene: 372360 Human

Unigene: 176725 Mouse

Unigene: 92828 Rat



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

BBS9是一种887个氨基酸的蛋白质,定位于细胞质和中心体,并以六个交替剪接的亚型存在。BBS9在多种组织中表达,包括肝、肺、心脏、脑和骨骼肌,BSS9作为纤毛虫发生所需的多蛋白BBAO复合物的一个组成部分,受到GDP/GTP交换因子的调节。BBS9基因的缺陷与BARDET BieDL综合征9型(BBS9)的发病机制有关,BAD是一种常染色体隐性遗传疾病,其特征是严重的色素性视网膜病变、早发性肥胖、多指畸形、低生育期、肾畸形和智力低下。此外,涉及BBS9基因的染色体畸变可能在肾母细胞瘤5(WT5)的形成中发挥作用。

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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