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货号： bs-8091R-RBITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-8091R-RBITC
- 英文名称
- Anti-CCDC40/RBITC
- 中文名称
- 罗丹明（RBITC）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.