| 產(chǎn)品編號(hào) | bs-8091R | 
| 英文名稱 | CCDC40 Rabbit pAb | 
| 中文名稱 | 卷曲螺旋結(jié)構(gòu)域蛋白40抗體 | 
| 別 名 | CCD40_HUMAN; ccdc 40; CCDC40; Coiled coil domain containing 40; coiled-coil domain containing 40; Coiled-coil domain-containing protein 40. | 
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) 表觀遺傳學(xué) | 
| 抗體來源 | Rabbit | 
| 克隆類型 | Polyclonal | 
| 克 隆 號(hào) | |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rat,Pig,Sheep,Cow,Dog,Horse) | 
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ELISA=1:5000-10000
 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.  | 
| 理論分子量 | 130 kDa | 
| 檢測(cè)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 CCDC40: 851-950/1142 | 
| 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 | 
Required for assembly of dynein regulatory complex (DRC) and inner dynein arm complexes, which are responsible for ciliary beat regulation, thereby playing a central role in motility in cilia and flagella. Not required for outer dynein arm complexes assembly. Required for axonemal recruitment of CCDC39. Involvement in disease: Defects in CCDC40 are the cause of primary ciliary dyskinesia type 15 (CILD15) . A disorder characterized by 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 randomization of left-right body asymmetry and situs inversus, due to dysfunction of monocilia at the embryonic node. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome. Function: Required for assembly of dynein regulatory complex (DRC) and inner dynein arm complexes, which are responsible for ciliary beat regulation, thereby playing a central role in motility in cilia and flagella. Not required for outer dynein arm complexes assembly. Required for axonemal recruitment of CCDC39. Subcellular Location: Cytoplasm. Cell projection, cilium. Note=Localizes to cytoplasm and motile cilium. DISEASE: Defects in CCDC40 are the cause of primary ciliary dyskinesia type 15 (CILD15) [MIM:613808]. A disorder characterized by 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 randomization of left-right body asymmetry and situs inversus, due to dysfunction of monocilia at the embryonic node. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome. Similarity: Belongs to the CCDC40 family. SWISS: Q4G0X9 Gene ID: 55036 Database links: Entrez Gene: 55036 Human Omim: 613799 Human SwissProt: Q4G0X9 Human Unigene: 202542 Human  |