结蛋白单克隆抗体

商品属性

产品介绍
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

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.

靶点与功能

结蛋白是III类中间丝蛋白，分子量约53kDa，仅表达于骨骼肌、心肌和平滑肌细胞，是肌肉细胞骨架的核心成分。其核心功能包括：

结构支撑：连接肌原纤维Z带与质膜，维持肌细胞形态和收缩稳定性；

疾病关联：突变或表达异常与结蛋白相关肌病（Desminopathy）、扩张型心肌病、肌营养不良密切相关；

临床价值：通过IHC检测可辅助鉴别横纹肌来源肿瘤（如横纹肌肉瘤）与其他软组织肿瘤，是病理诊断的“金标准”标志物。

实验操作关键要点

IHC-P优化（心肌组织切片）：

抗原修复：采用柠檬酸钠缓冲液（pH6.0） 微波修复20分钟（95℃），增强胞质信号；

阳性定位：心肌细胞胞质呈细丝状或网格状棕黄色阳性，Z带区域信号集中；

对照设置：必须包含骨骼肌组织（强阳性对照）和肝脏组织（阴性对照）。

WB检测注意事项：

样本处理：使用含1mM PMSF的RIPA裂解液冰上裂解30分钟，12000g离心取上清（结蛋白易被蛋白酶降解）；

电泳条件：10% SDS-PAGE凝胶，恒压80V浓缩胶、120V分离胶，目标条带约53kDa；

抗体孵育：抗体1:1000稀释（5% BSA+TBST），4℃摇床孵育过夜，HRP二抗（山羊抗兔）1:5000室温孵育1小时。

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