Anti-Desmin Polyclonal Antibody
- 發(fā)布日期: 2019-05-13
- 更新日期: 2025-07-11
產(chǎn)品詳請
產(chǎn)地 |
北京
|
品牌 |
Solarbio
|
保存條件 |
Store at -20°C. Avoid freeze / thaw cycles.
|
貨號 |
K106550P
|
應(yīng)用范圍 |
WB
|
CAS編號 |
|
抗體名 |
Anti-Desmin Polyclonal Antibody
|
克隆性 |
Polyclonal Antibody
|
靶點 |
|
適應(yīng)物種 |
Human Mouse Rat
|
形態(tài) |
|
宿主 |
Rabbit
|
亞型 |
IgG
|
標(biāo)識物 |
CSM1;CSM2;LGMD2R;DES;Desmin
|
濃度 |
%
|
免疫原 |
A synthetic peptide of human Desmin
|
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.