| 產(chǎn)品編號 | bs-11472R-BF594 |
| 英文名稱(chēng) | Rabbit Anti-RSPH4A/BF594 Conjugated antibody |
| 中文名稱(chēng) | BF594標記的Kartagener綜合征相關(guān)蛋白RSHL3抗體 |
| 別 名 | CILD11; dJ412I7.1; Radial spoke head protein 4 homolog A; Radial spoke head-like protein 3; RSH4A_HUMAN; RSHL3; Rsph4a; RSPH6B; A230081C05. |
| 規格價(jià)格 | 100ul/2980元 購買(mǎi) 大包裝/詢(xún)價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 研究領(lǐng)域 | 細胞生物 發(fā)育生物學(xué) 神經(jīng)生物學(xué) 信號轉導 細胞骨架 細胞外基質(zhì) |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 交叉反應 | |
| 產(chǎn)品應用 | 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 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 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 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 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. |
| 產(chǎn)品介紹 |
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綜合征:由下列三聯(lián)癥組成，支氣管擴張、鼻竇炎或鼻息肉及內臟反位(主要是右位心）。若僅具備內臟反位及支氣管擴張兩項，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、黏液纖毛運輸功能障礙，分泌物和細菌潴留而發(fā)生持續性感染長(cháng)期存在所致。以學(xué)齡兒童及青少年多發(fā)，有家族史。主要癥狀為隨年齡加重的咳嗽、咳痰和咯血，晨起明顯，易患感冒及肺炎，常見(jiàn)體征為發(fā)紺和杵狀指。 |
