Kartagener综合征相关蛋白RSHL3抗体

本公司是最专业Kartagener综合征相关蛋白RSHL3抗体供应商，提供Kartagener综合征相关蛋白RSHL3抗体的报价，泛素样蛋白Sumo2/3抗体咨询，

英文名称  Anti-RSPH4A/RSHL3

中文名称  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.

浓    度  1mg/1ml

规 格  0.2ml/200μg

Kartagener综合征相关蛋白RSHL3抗体概述：

B淋巴细胞在抗原的刺激下，能够分化、增殖形成具有针对这种抗原分泌特异性抗体的能力。B细胞的这种能力和量是有限的，不可能持续分化增殖下去，因此产生免疫球蛋白的能力也是极其微小的。将这种B细胞与非分泌型的骨髓瘤细胞融合形成杂交瘤细胞，再进一步克隆化，这种克隆化的杂交瘤细胞是既具有瘤细胞的无限分裂的能力，又具有产生特异性抗体的B淋巴细胞的能力。将这种克隆化的杂交瘤细胞进行培养或注入小鼠腹水内即可获得大量的高效、单一的特异性抗体。这种技术即称为单克隆抗体技术。

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

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综合征相关蛋白RSHL3抗体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咳嗽、咳痰和咯血，晨起明显，易患感冒及肺炎，常见体征为发绀和杵状指。