产品属性：

商品介绍：

英文名称Desmin Mouse mAb

中文名称结蛋白单克隆抗体

别    名DESM_HUMAN; DES;  

研究领域肿瘤  心血管  免疫学  信号转导  

抗体来源Mouse

克隆类型Monoclonal

克 隆 号3B12

交叉反应Human,Mouse,Rat

产品应用WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

理论分子量52 kDa

检测分子量

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 

亚    型IgG

纯化方法affinity purified by Protein G

缓 冲 液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

保存条件Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.

注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

PubMedPubMed

Desmin Mouse mAb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.