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Myotilin 抗体 (AA 121-200) (Cy5.5)

This anti-Myotilin antibody (ABIN1415400) is a Rabbit Polyclonal antibody detecting Myotilin in IF (cc), IF (p). Suitable for Human.
产品编号 ABIN1415400
发货至: 中国

Quick Overview for Myotilin 抗体 (AA 121-200) (Cy5.5) (ABIN1415400)

抗原

See all Myotilin (MYOT) 抗体
Myotilin (MYOT)

适用

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宿主

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克隆类型

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多克隆

标记

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This Myotilin antibody is conjugated to Cy5.5

应用范围

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Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
  • 抗原表位

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    AA 121-200

    预测反应

    Human,Mouse,Rat,Dog,Cow,Pig,Horse

    纯化方法

    Purified by Protein A.

    免疫原

    KLH conjugated synthetic peptide derived from human Myotilin

    亚型

    IgG
  • 应用备注

    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200

    限制

    仅限研究用
  • 状态

    Liquid

    浓度

    1 μg/μL

    缓冲液

    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

    储存液

    ProClin

    注意事项

    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

    储存条件

    -20 °C

    储存方法

    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

    有效期

    12 months
  • 抗原

    Myotilin (MYOT)

    别名

    Myotilin

    背景

    Synonyms: 57 kDa cytoskeletal protein, LGMD 1, LGMD1, Myofibrillar titin like Ig domains protein, Myofibrillar titin-like Ig domains protein, MYOT, MYOTI_HUMAN, Myotilin, Titin immunoglobulin domain protein, TTID, TTID protein.

    Background: Myotilin, a sarcomeric protein that is encoded by the gene mapping to human chromosome 5q31, binds to a-actinin and is 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 adult 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.

    基因ID

    9499
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