货品编号： bioss.bs-1026R 品 牌：bioss 品 名：Rabbit Anti-Desmin antibody 规 格： 50ul 产品描述： 研究领域 肿瘤心血管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,Chicken,Dog,Pig,Cow,Horse,Rabbit,Sheep,Guinea Pig, 产品应用 ELISA=1:500-1000IHC-P=1:100-500IHC-F=1:100-500Flow-Cyt=1μg/Test（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 52kDa 细胞定位 细胞浆 性状 Liquid 浓度 1mg/ml 免疫原 KLH conjugated synthetic peptide derived from human Desmin:261-360/470 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 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. 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.