| 產(chǎn)品編號 | bs-11472R |
| 英文名稱 | RSPH4A Rabbit pAb |
| 中文名稱 | 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. |
| 研究領(lǐng)域 | 細(xì)胞生物 發(fā)育生物學(xué) 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo) 細(xì)胞骨架 細(xì)胞外基質(zhì) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應(yīng) | Human,Mouse (predicted: Rat,Pig,Sheep,Cow,Chicken,Dog,Horse) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 81 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human RSPH4A/RSHL3: 435-482/716 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 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. SWISS: Q5TD94 Gene ID: 345895 Database links: Entrez Gene: 345895 Human Omim: 612647 Human SwissProt: Q5TD94 Human Unigene: 160380 Human Kartagener綜合征:由下列三聯(lián)癥組成，支氣管擴(kuò)張、鼻竇炎或鼻息肉及內(nèi)臟反位(主要是右位心）。若僅具備內(nèi)臟反位及支氣管擴(kuò)張兩項(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，常見體征為發(fā)紺和杵狀指。 |
| 產(chǎn)品圖片 |
Sample:
Trachea (Mouse) Lysate at 40 ug
Primary: Anti-RSPH4A? (bs-11472R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 81 kD
Observed band size: 81 kD
Sample:
Lung (Mouse) Lysate at 40 ug
A431(Human) Cell Lysate at 30 ug
Testis (Mouse) Lysate at 40 ug
Primary: Anti-RSPH4A? (bs-11472R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 81 kD
Observed band size: 81 kD
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