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Anti-Desmin Polyclonal Antibody
產(chǎn)品簡(jiǎn)介:

Anti-Desmin Polyclonal Antibody
應(yīng)用 WB
稀釋比例WB 1:3000.
交叉反應(yīng)Human Mouse Rat

產(chǎn)品型號(hào):K106550P

更新時(shí)間:2022-08-15

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Anti-Desmin Polyclonal Antibody

英文名稱Anti-Desmin Polyclonal Antibody
宿主Rabbit
別名CSM1;CSM2;LGMD2R;DES;Desmin
應(yīng)用WB
稀釋比例WB 1:3000.
交叉反應(yīng)Human Mouse Rat
蛋白分子量65kDa
Gene ID1674
保存Store at -20°C. Avoid freeze / thaw cycles.
儲(chǔ)存液Buffer: PBS with 0.03% Proclin300, 50% glycerol, pH7.3.
純化方法Affinity purification
亞型IgG
免疫原A synthetic peptide of human Desmin
性狀液體
Public Immunogen RangeA synthetic peptide of human Desmin
Subcellular LocationsCell membrane Cytoplasm sarcolemma
Swiss ProtP17661
克隆類型Polyclonal Antibody
背景資料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.

 

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