Anti-CCDC39抗体Required for assembly of dynein regulatory complex （DRC） and inner dynein arm complexes, which are responsible for ciliary beat regulation,

产品编号 yb-8096R
英文名称 Anti-CCDC39抗体
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.
Anti-CCDC39抗体 
说 明 书 0.2ml  
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, Mouse, Rat, Cow, Horse, Rabbit, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 110kDa
细胞定位 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human CCDC39
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.

PubMed PubMed
产品介绍 background:
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. 


Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.