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Myotilin 抗体 (AA 121-200)

MYOT 适用: 人 ELISA, IF (p), IF (cc), IHC (p), IHC (fro) 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN1386097
发货至: 中国
  • 抗原 See all Myotilin (MYOT) 抗体
    Myotilin (MYOT)
    抗原表位
    • 14
    • 7
    • 6
    • 6
    • 6
    • 3
    • 2
    • 1
    • 1
    • 1
    AA 121-200
    适用
    • 46
    • 6
    • 4
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    宿主
    • 43
    • 2
    • 1
    克隆类型
    • 45
    • 1
    多克隆
    标记
    • 16
    • 3
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This Myotilin antibody is un-conjugated
    应用范围
    • 25
    • 19
    • 12
    • 12
    • 6
    • 4
    • 4
    • 3
    • 1
    • 1
    ELISA, Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunofluorescence (Cultured Cells) (IF (cc)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))
    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
    Top Product
    Discover our top product MYOT Primary Antibody
  • 应用备注
    ELISA 1:500-1000
    IHC-P 1:200-400
    IHC-F 1:100-500
    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
    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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
    4 °C,-20 °C
    储存方法
    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    有效期
    12 months
  • 抗原
    Myotilin (MYOT)
    别名
    Myotilin (MYOT 产品)
    别名
    LGMD1 antibody, LGMD1A antibody, MFM3 antibody, TTID antibody, TTOD antibody, 5530402I04Rik antibody, Ttid antibody, MYOT antibody, ttid antibody, myotilin antibody, MYOT antibody, Myot antibody, myot antibody
    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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