卷曲螺旋结构域蛋白40抗体
规格：1尘驳/1尘濒
英文名： CCDC40
别名： CCD40_HUMAN; ccdc 40; CCDC40; Coiled coil domain containing 40; coiled-coil domain containing 40; Coiled-coil domain-containing protein 40.
分子量： 130kDa
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 CCDC40
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,
细胞定位：细胞浆
卷曲螺旋结构域蛋白40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. 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卷曲螺旋结构域蛋白40抗体 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, 卷曲螺旋结构域蛋白40抗体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. Gene ID: 55036 Database links: Entrez Gene: 55036 Human Omim: 613799 Human SwissProt: Q4G0X9 Human Unigene: 202542 Human 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40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 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid