Anti-Desmin Polyclonal Antibody-一抗-抗体-蛋白与免疫

Anti-Desmin Polyclonal Antibody

品牌:solarbio | 货号:K106550P

英文名称 Anti-Desmin Polyclonal Antibody
宿主 Rabbit
别名 CSM1;CSM2;LGMD2R;DES;Desmin
应用 WB
稀释比例 WB 1:3000.
交叉反应 Human Mouse Rat
蛋白分子量 65kDa
Gene ID 1674
保存 Store at -20°C. Avoid freeze / thaw cycles.
储存液 Buffer: PBS with 0.03% Proclin300, 50% glycerol, pH7.3.
纯化方法 Affinity purification
亚型 IgG
免疫原 A synthetic peptide of human Desmin
性状 液体
Public Immunogen Range A synthetic peptide of human Desmin
Subcellular Locations Cell membrane Cytoplasm sarcolemma
Swiss Prot P17661
克隆类型 Polyclonal Antibody
背景资料 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.