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| 规格: | 50ul | 产品价格: | ¥1180.0 |
|---|---|---|---|
| 规格: | 100ul | 产品价格: | ¥1980.0 |
| 规格: | 200ul | 产品价格: | ¥2800.0 |
| 产品编号 | bs-11472R |
| 英文名称 | RSPH4A Rabbit pAb |
| 中文名称 | Kartagener综合征相关蛋白RSHL3抗体 |
| 英文别名 | CILD11; dJ412I7.1; Radial spoke head protein 4 homolog A; Radial spoke head-like protein 3; RSH4A_HUMAN; RSHL3; Rsph4a; RSPH6B; A230081C05. |
| 产品应用 | WB=1:500-2000 Not yet tested in other applications. |
| 交叉反应 | Human, Mouse (Rat, Chicken, Dog, Pig, Cow, Horse, Sheep) |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human RSPH4A/RSHL3 |
| 亚型 | IgG |
| 性状 | Liquid |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 理论分子量 | 81 kDa |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Neuroscience > Neurology process > Growth and Development > Axonal Guidance Proteins Signal Transduction > Cytoskeleton / ECM > Cytoskeleton > Motor Proteins > Dynein |
| 亚细胞定位 | Cytoplasm; cytoskeleton; cilium axoneme. Radial spoke. |
| 组织特异性 | 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. |
| 相似性 | Belongs to the flagellar radial spoke RSP4/6 family. |
| 功能 | 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. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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. |
| 应用 | 推荐稀释比例 |
| {WB} | {1:500-2000} |

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

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
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