耳聋-甲状腺肿综合征相关COBL蛋白抗体

耳聋-甲状腺肿综合征相关COBL蛋白抗体

英文名称    COBL    

中文名称    耳聋-甲状腺肿综合征相关COBL蛋白抗体    

别    名    cobL; COBL_HUMAN; DKFZp686G13227; KIAA0633; MGC131893; Protein cordon-bleu.      

研究领域    心血管  细胞生物  免疫学  发育生物学  神经生物学      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Dog, Horse,     

产品应用    WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=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    136kDa    

细胞定位    细胞浆 细胞膜     

性    状    Lyophilized or Liquid    

浓    度    1mg/ml    

免 疫 原    KLH conjugated synthetic peptide derived from human COBL:181-280/1261     

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    Cordon-bleu, also known as COBL, is a 1,261 amino acid protein that localizes to the node of the axial midline, a structure that organizes morphogenesis of the vertebrate embryo. Widely conserved and existing as five alternatively spliced isoforms, Cordon-bleu interacts with Vangl2 to mediate closure of the midbrain neural tube and is highly expressed in pancreas, ovary, brain, liver, lung and kidney. Cordon-bleu contains three WH2 domains and is encoded by a gene that maps to human chromosome 7, which houses over 1,000 genes and comprises nearly 5% of the human genome. Chromosome 7 has been linked to Osteogenesis imperfecta, Pendred syndrome, Lissencephaly, Citrullinemia and Shwachman-Diamond syndrome. The deletion of a portion of the q arm of chromosome 7 is associated with Williams-Beuren syndrome, a condition characterized by mild mental retardation, an unusual comfort and friendliness with strangers and an elfin appearance.