Myotilin 抗体 (AA 121-200) (FITC)
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- 抗原 See all Myotilin (MYOT) 抗体
- Myotilin (MYOT)
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抗原表位
- AA 121-200
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适用
- 人
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This Myotilin antibody is conjugated to FITC
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应用范围
- Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunofluorescence (Cultured Cells) (IF (cc))
- 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
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- 应用备注
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - 限制
- 仅限研究用
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- 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
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- 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
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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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