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RabbitAnti-CCDC39 Rabbit pAb  antibody (bs-8096R)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-8096R
英文名称 CCDC39 Rabbit pAb
中文名称 卷曲螺旋结构域蛋白39抗体
别    名 CCD39_HUMAN; Ccdc39; Coiled-coil domain-containing protein 39.  
研究领域 细胞生物  免疫学  信号转导  细胞骨架  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,  (predicted: Mouse, Rat, Cow, Horse, Rabbit, )
产品应用 WB=1:500-2000 ELISA=1:5000-10000 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 110 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CCDC39: 651-750/941 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
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.
PubMed PubMed
产品介绍 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.
Tissue specificity:Mainly expressed in nasal brushings and, to a lesser extent, in lungs and testis.
Involvement in disease:
Defects in CCDC39 are the cause of primary ciliary dyskinesia type 14 (CILD14) . 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.

Subcellular Location:
Cytoplasm, cytoskeleton, cilium axoneme. Note=CCDC40 is required for localization to axonemes.

Tissue Specificity:
Mainly expressed in nasal brushings and, to a lesser extent, in lungs and testis.

DISEASE:
Defects in CCDC39 are the cause of primary ciliary dyskinesia type 14 (CILD14) [MIM:613807]. 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 CCDC39 family.

SWISS:
Q9UFE4

Gene ID:
339829

Database links:

Entrez Gene: 488089 Dog

Entrez Gene: 339829 Human

Entrez Gene: 51938 Mouse

Omim: 613798 Human

SwissProt: E2R1I5 Dog

SwissProt: Q9UFE4 Human

SwissProt: Q9D5Y1 Mouse

Unigene: 712820 Human

Unigene: 474546 Mouse



产品图片
Sample: DU145(Human) Cell Lysate at 30 ug Primary: Anti- CCDC39 (bs-8096R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 110 kD Observed band size: 110 kD
Sample: HL-60(Human) Cell Lysate at 30 ug Primary: Anti- CCDC39 (bs-8096R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 110 kD Observed band size: 110 kD
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