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產(chǎn)品詳情
  • 產(chǎn)品名稱(chēng):Anti-Myotilin抗體

  • 產(chǎn)品型號(hào):肌收縮蛋白MYOT抗體
  • 產(chǎn)品廠商:KALANG
  • 產(chǎn)品文檔:
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簡(jiǎn)單介紹:
Anti-Myotilin抗體產(chǎn)品質(zhì)量穩(wěn)定,實(shí)驗(yàn)效果明顯,貨期快,價(jià)格優(yōu)惠,歡迎垂詢(xún)訂購(gòu)!我公司長(zhǎng)期供應(yīng)**組化抗體、WB抗體、**組化試劑盒和抗體試驗(yàn)所需全部相關(guān)試劑、熒光標(biāo)記抗體、單克隆抗體、多克隆抗體、各種標(biāo)記的二抗IgG/IgM/IgD/IgA等科研實(shí)驗(yàn)抗體。Anti-Myotilin抗體用于**組化實(shí)驗(yàn),WB實(shí)驗(yàn),相應(yīng)的標(biāo)記抗體有HRP標(biāo)記抗體,FITC標(biāo)記,BIO等。
詳情介紹:
Rabbit  Anti-Myotilin
Cat. Number:
Anti-Myotilin抗體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抗體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抗體 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:
Rabbit Polyclonal IgG, affinity purified by Protein A.
Reacts with: Human, Mouse, Rat, Anti-Myotilin抗體Dog, Pig, Cow, Horse, .
Immunogen: KLH conjugated synthetic peptide derived from human Myotilin.
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抗體
Optimal working dilutions must be determined by the end user.

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