FITC标记的轴丝动力蛋白11抗体上海沪震实业有限公司

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产品名称: FITC标记的轴丝动力蛋白11抗体
英文名称: Anti-DNAH11/FITC
抗体货号: HZ-14360R-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: 2018/10/12 9:36:00
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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上海·中国-上海
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产品详细描述

 Rabbit Anti-DNAH11/FITC Conjugated antibody 

FITC标记的轴丝动力蛋白11抗体

 

英文名称 Anti-DNAH11/FITC
中文名称 FITC标记的轴丝动力蛋白11抗体
别    名 axonemal; Axonemal beta dynein heavy chain 11; axonemal, heavy polypeptide 11; CILD7; Ciliary dynein heavy chain 11; DNAH11; DNAHBL; DNHBL; DPL11; DYH11_HUMAN; Dynein heavy chain 11; dynein, ciliary, heavy chain 11.  
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  信号转导  细胞骨架  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, 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 521kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DNAH11
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:
This gene encodes a ciliary outer dynein arm protein and is a member of the dynein heavy chain family. It is a microtubule-dependent motor ATPase and has been reported to be involved in the movement of respiratory cilia. Mutations in this gene have been implicated in causing Kartagener Syndrome (a combination of situs inversus totalis and Primary Ciliary Dyskinesia (PCD), also called Immotile Cilia Syndrome 1 (ICS1)) and male sterility. [provided by RefSeq, Mar 2013]

Function:
Force generating protein of respiratory cilia. Produces force towards the minus ends of microtubules. Dynein has ATPase activity; the force-producing power stroke is thought to occur on release of ADP.

Subcellular Location:
Cytoplasm; cytoskeleton; cilium axoneme.

DISEASE:
Defects in DNAH11 are a cause of Kartagener syndrome (KTGS) [MIM:244400]. KTGS is an autosomal recessive disorder characterized by the association of primary ciliary dyskinesia with situs inversus. Clinical features include recurrent respiratory infections, bronchiectasis, infertility, and lateral transposition of the viscera of the thorax and abdomen. The situs inversus is most often total, although it can be partial in some cases (isolated dextrocardia or isolated transposition of abdominal viscera). Defects in DNAH11 are the cause of primary ciliary dyskinesia type 7 (CILD7) [MIM:611884]. 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 dynein heavy chain family.

Database links:

Entrez Gene: 8701 Human

Omim: 603339 Human

SwissProt: Q96DT5 Human

Unigene: 520245 Human



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

该基因编码纤毛外动力蛋白臂蛋白,是Dynin重链家族的成员。它是一种微管依赖性的运动ATP酶,据报道参与了呼吸纤毛的运动。该基因的突变与Kar.ner综合征(全反位和原发性睫状肌营养不良(PCD)的结合,也称为不动性睫状肌综合征1(ICS1))和男性不育有关。〔RefSeq,2013〕

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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