产品详情
简单介绍：
Anti-Myotilin抗体产品质量稳定，实验效果明显，货期快，价格优惠，欢迎垂询订购！我公司长期供应**组化抗体、WB抗体、**组化试剂盒和抗体试验所需全部相关试剂、荧光标记抗体、单克隆抗体、多克隆抗体、各种标记的二抗IgG/IgM/IgD/IgA等科研实验抗体。Anti-Myotilin抗体用于**组化实验,WB实验,相应的标记抗体有HRP标记抗体,FITC标记,BIO等。
详情介绍：
Rabbit Anti-Myotilin
Cat. Number:
Anti-Myotilin抗体KL-6479R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
Myotilin, a sarcomeric protein that is encoded by the gene mapping to human chromosome 5q31, binds to a-actinin and is
Anti-Myotilin抗体localized in the Z-line of myofibrils. Myotilin is expressed in skeletal and cardiac muscle, and it co-localizes with a-actinin in the sarcomeric I-bands where it directly interacts with a-actinin. Defects in the myotilin gene are reported to cause a form of autosomal dominant limb-girdle muscular dystrophy (LGMD). Symptoms of ***** onset LGMD are progressive weakness of the hip and shoulder girdles as well as a distinctive dysarthric pattern of speech. The muscle of affected individuals with LGMD shows degeneration of myofibers, variations in fiber size, fiber splitting, centrally located myonuclei and an enhanced number of autophagic vesicles.
Also known as:
57 kDa cytoskeletal protein; LGMD 1;
Anti-Myotilin抗体LGMD1; Myofibrillar titin like Ig domains protein; Myofibrillar titin-like Ig domains protein; MYOT; MYOTI_HUMAN; Myotilin; Titin immunoglobulin domain protein; TTID; TTID protein.
Specificity:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat,
Anti-Myotilin抗体Dog, Pig, Cow, Horse, .
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Immunogen: KLH conjugated synthetic peptide derived from human Myotilin.
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Predicted Molecular Weight: 55kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200
Not yet tested in other applications. Anti-Myotilin抗体
Optimal working dilutions must be determined by the end user.
Not yet tested in other applications. Anti-Myotilin抗体
Optimal working dilutions must be determined by the end user.
