结蛋白抗体

结蛋白抗体

英文名称    Desmin    

中文名称    结蛋白抗体    

别    名    CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793.      

研究领域    肿瘤  细胞生物  免疫学  信号转导  细胞类型标志物      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,     

产品应用    WB=1:500-2000 Flow-Cyt=1μg/Test  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    52kDa    

细胞定位    细胞浆     

性    状    Lyophilized or Liquid    

浓    度    1mg/ml    

免 疫 原    KLH conjugated synthetic peptide derived from human Desmin:401-470/470     

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    filaments found in muscle cells. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z line structures. Defects in Desmin are the cause of desmin related cardio skeletal myopathy (CSM) also known as desmin related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin reactive deposits in cardiac and skeletal muscle cells. A desmin related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Antidesmin antibodies are useful in identification of tumours of myogenic origin.    

Desmin在很多哺乳动物中的横纹肌和各种平滑肌及其来源的肿瘤组织中都有表达。结蛋白是一种中间丝蛋白，广泛分布于骨骼肌细胞、平滑肌细胞、心肌细胞和肌上皮细胞及其肿瘤中，主要用于子宫、皮肤、胃肠道及其它横纹肌肉瘤和肌上皮瘤的诊断和鉴别诊断。