FITC标记的Kartagener综合征相关蛋白RSHL3抗体上海沪震实业有限公司

性能参数

产品名称: FITC标记的Kartagener综合征相关蛋白RSHL3抗体
英文名称: Anti-RSPH4A/FITC
抗体货号: HZ-11472R-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 9:50:00
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-RSPH4A/FITC Conjugated antibody

FITC标记的Kartagener综合征相关蛋白RSHL3抗体

 

产品编号 bs-11472R-FITC
英文名称 Anti-RSPH4A/FITC
中文名称 FITC标记的Kartagener综合征相关蛋白RSHL3抗体
别    名 CILD11; dJ412I7.1; Radial spoke head protein 4 homolog A; Radial spoke head-like protein 3; RSH4A_HUMAN; RSHL3; Rsph4a; RSPH6B; A230081C05.  
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  发育生物学  神经生物学  信号转导  细胞骨架  细胞外基质  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, 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 81kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human RSPH4A/RSHL3 (435-482aa)
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:
RSHL3 is predicted to be a component of the radial spoke head based on homology with proteins in the biflagellate alga Chlamydomonas reinhardtii and other ciliates. RSHL3 (radial spoke head-like protein 3), also known as radial spoke head protein 4 homolog A, is a 716 amino acid protein that belongs to the flagellar radial spoke RSP4/6 family. Mutations in the RSHL3 gene cause primary ciliary dyskinesia 1, a disease arising from dysmotility of motile cilia and sperm. Existing as three alternatively spliced isoforms, the RSHL3 gene contains 6 exons, is conserved in chimpanzee, dog, cow, mouse, rat, chicken, zebrafish, fruit fly and P.falciparum, and maps to human chromosome 6q22.1.

Function:
Probable component of the axonemal radial spoke head. Radial spokes are regularly spaced along cilia, sperm and flagella axonemes. They consist of a thin stalk which is attached to a subfiber of the outer doublet microtubule, and a bulbous head which is attached to the stalk and appears to interact with the projections from the central pair of microtubules.

Subcellular Location:
Cytoplasm; cytoskeleton; cilium axoneme. Radial spoke.

Tissue Specificity:
Defects in RSPH4A are the cause of primary ciliary dyskinesia type 11 (CILD11) [MIM:612649]. CILD is an autosomal recessive disorder characterized by axonemal abnormalities of motile cilia. Respiratory infections leading to chronic inflammation and bronchiectasis are recurrent, due to defects in the respiratory cilia; reduced fertility is often observed in male patients due to abnormalities of sperm tails. Half of the patients exhibit situs inversus, due to dysfunction of monocilia at the embryonic node and randomization of left-right body asymmetry. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome.

DISEASE:
Defects in RSPH4A are the cause of primary ciliary dyskinesia type 11 (CILD11) [MIM:612649]. CILD is an autosomal recessive disorder characterized by axonemal abnormalities of motile cilia. Respiratory infections leading to chronic inflammation and bronchiectasis are recurrent, due to defects in the respiratory cilia; reduced fertility is often observed in male patients due to abnormalities of sperm tails. Half of the patients exhibit situs inversus, due to dysfunction of monocilia at the embryonic node and randomization of left-right body asymmetry. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome. 

Similarity:
Belongs to the flagellar radial spoke RSP4/6 family.

Database links:

Entrez Gene: 345895 Human

Omim: 612647 Human

SwissProt: Q5TD94 Human

Unigene: 160380 Human



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

Kartagener综合征:由下列三联症组成,支气管扩张、鼻窦炎或鼻息肉及内脏反位(主要是右位心)。若仅具备内脏反位及支气管扩张两项,则为不全性Kartagener综合征。常合并其他先天性畸形。其病因是由于全身纤毛先天性缺乏轴丝臂,引起纤毛活动力丧失、黏液纤毛运输功能障碍,分泌物和细菌潴留而发生持续性感染长期存在所致。以学龄儿童及青少年多发,有家族史。主要症状为随年龄加重的**、*痰和咯血,晨起明显,易患感冒及肺炎,常见体征为发绀和杵状指
   

RSL3是基于双鞭藻藻衣藻和其他纤毛虫的蛋白质同源性预测的辐辐头的一个组成部分。RSL3(辐状头状蛋白3),又称辐辐头蛋白4同系物A,是属于鞭毛辐辐RSP4/6家族的716氨基酸蛋白质。RSHL3基因的突变引起原发性睫状体运动障碍1,一种由活动纤毛和精子的运动障碍引起的疾病。RSL3基因作为三个可替代剪接的亚型存在,包含6个外显子,在黑猩猩、狗、牛、小鼠、大鼠、鸡、斑马鱼、果蝇和恶性疟原虫中保存,并映射到人类染色体6q22.1。

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Kartagener综合征相关蛋白RSHL3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