“結蛋白抗體,Anti-Desmin/DES抗體”應用于IHC、WB、 IF、IP、ELISA，質量保證，無效免費退換，另外本公司生產(chǎn)經(jīng)營各種標記二抗、免疫印跡常用試劑、免疫組化常用試劑，咨詢！

產(chǎn)品介紹：

【產(chǎn)品名稱】：結蛋白抗體,Anti-Desmin/DES抗體

【產(chǎn)品類型】：一抗

【產(chǎn)品編號】：BYk-1026R                    

【相關標記】: HRP Biotin Gold RBITC AP FITC Cy3 Cy5 Cy5.5 Cy7 PE PE-Cy3 PE-CY5 PE-CY5.5 PE-CY7 APC Alexa Fluor 350 Alexa Fluor 488 Alexa Fluor 555 Alexa Fluor 647

【性 狀】： Lyophilized or Liquid

【濃 度】： 1mg/1ml

【亞 型】： IgG

【規(guī) 格】：0.1ml/100ug，0.2ml/200ug

【抗體來源】： Rabbit OR MOUSE

【克隆類型】： polyclonal or monoclonal

【產(chǎn)品用途】：科研實驗，用于免疫組化實驗，WB實驗、IF、IP、ELISA實驗，相應的標記抗體有HRP標記抗體，F(xiàn)ITC標記，BIO等。

【貯 存】： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

【信息詳情】： 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.

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