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货号： bs-8096R-RBITC 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-8096R-RBITC
- 英文名称
- Anti-CCDC39/RBITC
- 中文名称
- 罗丹明（RBITC）标记的卷曲螺旋结构域蛋白39抗体
- 别 名
- CCD39_HUMAN; Ccdc39; Coiled-coil domain-containing protein 39.
- 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 信号转导 细胞骨架
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Cow, Horse, Rabbit,
- 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
- 110kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human CCDC39
- 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.
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:
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. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome.
Similarity:
Belongs to the CCDC39 family.
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.