卷曲螺旋结构域蛋白39抗体
规格：1尘驳/1尘濒
英文名： CCDC39
别名： CCD39_HUMAN; Ccdc39; Coiled-coil domain-containing protein 39.
分子量： 110kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human CCDC39
交叉反应：Human, Mouse, Rat, Cow, Horse, Rabbit,
细胞定位：细胞浆
卷曲螺旋结构域蛋白39抗体产物介绍：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: 卷曲螺旋结构域蛋白39抗体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.卷曲螺旋结构域蛋白39抗体 Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome. Similarity: Belongs to the CCDC39 family. 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
卷曲螺旋结构域蛋白39抗体产物应用：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.
研究领域：细胞生物 **学 信号转导 细胞骨架
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid