Anti-CCDC8抗体This gene encodes a coiled-coil domain-containing protein. The encoded protein functions as a cofactor required for p53-mediated apoptosis following DNA damage,

产品编号 yb-8138R
英文名称 Anti-CCDC8抗体
中文名称 卷曲螺旋结构域蛋白8抗体
别    名 CCDC8; CCDC8_HUMAN; Coiled-coil domain-containing protein 8; DKFZp564K0322; ENSMUSG00000041117; MGC72567.
Anti-CCDC8抗体
说 明 书 0.2ml  
研究领域 细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Horse, 
产品应用 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.
分 子 量 59kDa
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human CCDC8
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.

PubMed PubMed
产品介绍 background:
This gene encodes a coiled-coil domain-containing protein. The encoded protein functions as a cofactor required for p53-mediated apoptosis following DNA damage, and may also play a role in growth through interactions with the cytoskeletal adaptor protein obscurin-like 1. Mutations in this gene are a cause of 3M syndrome-3 (3M3). [provided by RefSeq, Dec 2011].

Subunit:
Interacts with OBSL1. 

Tissue Specificity:
Widely expressed with low levels in spleen, skeletal muscle, small intestine, kidney and liver.

Post-translational modifications:
Phosphorylated upon DNA damage, probably by ATM or ATR. 

DISEASE:
Defects in CCDC8 are the cause of 3M syndrome type 3 (3M3) [MIM:614205]. A disorder characterized by poor postnatal growth and distinctive facial features, including triangular facies, frontal bossing, fleshy tipped nose, and fleshy lips. Other features may include skeletal anomalies and prominent heels. 

Database links:
UniProtKB/Swiss-Prot: Q9H0W5.2

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.