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Kartagener綜合征相關(guān)蛋白R(shí)SHL3抗體規(guī)格 英文名稱  Anti-RSPH4A/RSHL3

中文名稱  Kartagener綜合征相關(guān)蛋白R(shí)SHL3抗體規(guī)格 

別    名 CILD11; dJ412I7.1; Radial spoke head protein 4 homolog A; Radial spoke head-like protein 3; RSH4A_HUMAN; RSHL3; Rsph4a; RSPH6B; A230081C05.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.2ml/200μg

抗體來(lái)源  Rabbit

克隆類型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep

產(chǎn)品類型   一抗  

研究領(lǐng)域    細(xì)胞生物 發(fā)育生物學(xué) 神經(jīng)生物學(xué) 信號(hào)轉(zhuǎn)導(dǎo) 細(xì)胞骨架 細(xì)胞外基質(zhì)

蛋白分子量  predicted molecular weight: 81kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human RSPH4A/RSHL3 (435-482aa)

亞    型  IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

Kartagener綜合征相關(guān)蛋白R(shí)SHL3抗體規(guī)格 產(chǎn)品應(yīng)用   WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500

（石蠟切片需做抗原修復(fù)） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存條件  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. 

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

產(chǎn)品介紹 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 : UniProtKB/Swiss-Prot: Q5TD94.1

Kartagener綜合征:由下列三聯(lián)癥組成，、或及內(nèi)臟反位(主要是右位心）。若僅具備內(nèi)臟反位及兩項(xiàng)，則為不全性Kartagener綜合征。常合并其他先天性畸形。其病因是由于全身纖毛先天性缺乏軸絲臂，引起纖毛活動(dòng)力喪失、黏液纖毛運(yùn)輸功能障礙，分泌物和細(xì)菌潴留而發(fā)生持續(xù)性感染長(zhǎng)期存在所致。以學(xué)齡兒童及青少年多發(fā)，有家族史。主要為隨年齡加重的、和，晨起明顯，易患及，常見體征為和杵狀指。

全、新、優(yōu)、品、好四大特點(diǎn)：

Kartagener綜合征相關(guān)蛋白R(shí)SHL3抗體規(guī)格 全：公司提供上萬(wàn)種產(chǎn)品，涵蓋了生物試劑，elisa試劑盒，標(biāo)準(zhǔn)品，培養(yǎng)基，原裝耗材，抗體、培養(yǎng)基、ATCC細(xì)胞等，基本上各種科研所需產(chǎn)品在我司都能找到。

新：產(chǎn)品更新速度較快，基本上每周都有新產(chǎn)品出現(xiàn)。

優(yōu)：產(chǎn)品質(zhì)量好，投訴比較少。

好：我公司具有優(yōu)質(zhì)的技術(shù)團(tuán)隊(duì)，產(chǎn)品一旦售出，實(shí)驗(yàn)過(guò)程中遇到困難可提供在線技術(shù)咨詢。使您使用產(chǎn)品時(shí)沒有任何的后顧之憂。

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