Anti-CCDC40/FITC Conjugated抗体-抗体-抗体-生物在线
上海钰博生物科技有限公司
Anti-CCDC40/FITC Conjugated抗体

Anti-CCDC40/FITC Conjugated抗体

商家询价

产品名称: Anti-CCDC40/FITC Conjugated抗体

英文名称: Anti-CCDC40/FITC

产品编号: YB--8091R-FITC

产品价格: null

产品产地: 中国/美国

品牌商标: Ybscience

更新时间: 2023-08-17T10:29:50

使用范围: 科研使用

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 Anti-CCDC40/FITC Conjugated抗体

产品编号 YB-8091R-FITC
英文名称 Anti-CCDC40/FITC
中文名称 FITC标记的卷曲螺旋结构域蛋白40抗体
别    名 CCD40_HUMAN; ccdc 40; CCDC40; Coiled coil domain containing 40; coiled-coil domain containing 40; Coiled-coil domain-containing protein 40.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, 
产品应用 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 130kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CCDC40
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.
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. 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 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, 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. 

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