技术外包服务:

★Kartagener综合征相关蛋白RSHL3抗体规格 分子生物学：质粒抽提、PCR、Q-PCR、RT-PCR、分子生物学：基因合成、引物合成、基因测序、载体构建等

★蛋白工程：原核、哺乳动物蛋白表达系统等

★病毒包装：腺病毒、慢病毒等

★抗体工程：磁珠分选、病理染色、WB、ELISA、IP、IF、IHC、FACS、Confocal等等

★细胞工程：细胞表型分析(凋亡、增殖、周期、迁移、侵袭、修复、克隆形成)、细胞培养、细胞膜制备、稳定细胞株构建、细胞RNAi技术等等。

实验流程：

Kartagener综合征相关蛋白RSHL3抗体规格 英文名称  Anti-RSPH4A/RSHL3

中文名称  Kartagener综合征相关蛋白RSHL3抗体规格 

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

产品属性：

浓    度  1mg/1ml

规 格  0.2ml/200μg

抗体来源  Rabbit

克隆类型   polyclonal

交叉反应   Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep

产品类型   一抗  

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

蛋白分子量  predicted molecular weight: 81kDa 

性    状   Lyophilized or Liquid

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   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

Kartagener综合征相关蛋白RSHL3抗体规格 产品应用   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

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

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

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. 

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

产品介绍 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综合征:由下列三联症组成，、或及内脏反位(主要是右位心）。若仅具备内脏反位及两项，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。主要为随年龄加重的、和，晨起明显，易患及，常见体征为和杵状指。

全、新、优、品、好四大特点：

Kartagener综合征相关蛋白RSHL3抗体规格 全：公司提供上万种产品，涵盖了生物试剂，elisa试剂盒，标准品，培养基，原装耗材，抗体、培养基、ATCC细胞等，基本上各种科研所需产品在我司都能找到。

新：产品更新速度较快，基本上每周都有新产品出现。

优：产品质量好，投诉比较少。

好：我公司具有优质的技术团队，产品一旦售出，实验过程中遇到困难可提供在线技术咨询。使您使用产品时没有任何的后顾之忧。

正在热销的相关产品：

Beta-Amyloid(1-28)/FITC 荧光素标记β淀粉样肽(1-28)抗体IgGMulti-class antibodies规格： 0.2ml

Anti-FLAG Tag (NT)/FITC 荧光素标记抗标签FLAG Tag标签抗体(N端)IgGMulti-class antibodies规格： 0.2ml

Rhesus antibody Rh CXCL14 CXC趋化因子14抗体 规格 0.2ml

streptomycin/OVA 链霉素偶联鸡卵白蛋白 1mg

SETDB1 英文名称： 组蛋白H3K9甲基转移酶4抗体 0.2ml

Rhesus antibody Rh VTG 鱼、青鳉鱼卵黄蛋白原抗体 规格 0.2ml

Anti-FLAG Tag (NT)/FITC 荧光素标记抗标签FLAG Tag标签抗体(N端)IgGMulti-class antibodies规格： 0.2ml

Anti-phospho-PAK3(pSer139)/FITC 荧光素标记抗磷酸化p21激活激酶3抗体IgGMulti-class antibodies规格： 0.2ml

Goat Anti-Bovine IgG 羊抗IgG (亲和层析纯化)Multi-class antibodies规格： 1mg

抗体 anti-Measles virus of fusion protein 0.1ml

Goat Anti-rabbit IgG/APC APC标记的羊抗兔IgG 0.1ml

FMO3 英文名称： 二甲基苯胺单加氧酶3抗体 0.2ml

Rhesus antibody Rh Phospho-Stat2 (Tyr690) 磷酸化信号转导和转录激活因子2抗体 规格 0.1ml

Goat Anti-Bovine IgG 羊抗IgG (亲和层析纯化)Multi-class antibodies规格： 1mg

mouse Caspase-3 小鼠Caspase-3Multi-class antibodies规格： 48T

Anti-FLIPS 凋亡调节基因之一抗体(短型)Multi-class antibodies规格： 0.2ml

Rhesus antibody Rh Mouse Anti-rabbit IgG/PE-Cy5.5 PE-Cy5.5标记的小鼠抗兔IgG 规格 0.1ml

BLI(Human Beta-Lactamase inhibitors) ELISA Kit 人β内酰胺酶抑制剂 96T

Syntrophin-1 英文名称： 神经肌肉接头蛋白SNTA1抗体 0.2ml

CCDC68 英文名称： 卷曲螺旋结构域蛋白68抗体(皮肤T淋巴细胞相关抗原) 0.2ml

Anti-FLIPS 凋亡调节基因之一抗体(短型)Multi-class antibodies规格： 0.2ml

aClTrypticSoyBroth

K'smediumacidification

胰蛋白胨胆盐琼脂 Tryptone Bile Agar 250 用于大肠杆菌的膜过滤法选择性培养(ISO标准)

incubationmedia

3%NaClAlkalinePeptoneWater

XLDAgar

碱性蛋白胨水(APW) 1000(g) incubation media 碱性蛋白胨水(APW) 1000(g)

OF发酵管 OF发酵管 20支 BR

MH肉汤(MHB)250用于抗生素敏感试验incubationmediaMH肉汤(MHB)250用于抗生素敏感试验

细菌蛋白胨 250g 用于制备0.1%蛋白胨水，用于空肠弯曲菌的预增菌

Kartagener综合征相关蛋白RSHL3抗体规格 CATC琼脂 250(g)  incubation media CATC琼脂 250(g)

D/E中和琼脂 250(g)  incubation media D/E中和琼脂 250(g)

D/E中和肉汤 250(g)  incubation media D/E中和肉汤 250(g)

AC肉汤 250(g)  incubation media AC肉汤 250(g)