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Anti-CCDC39卷曲螺旋结构域蛋白39抗体
点击次数：168发布时间：2012/12/26 7:57:23
更新日期：2024/9/5 14:43:40
所 在 地：其它
产品型号：BY-8096R
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货号：BY-8096R
英文名称：Anti-CCDC39
中文名称：卷曲螺旋结构域蛋白39抗体
其他名称：CCD39_HUMAN;Ccdc39;Coiled-coil domain-containing protein 39.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 110kDa
纯化方法：affinity purified by Protein A
交叉反应：Human
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.
