Recombinant Dystrophin 抗体 (AA 1700-2300)
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Quick Overview for Recombinant Dystrophin 抗体 (AA 1700-2300) (ABIN7859275)
抗原
See all Dystrophin (DMD) 抗体抗体类型
适用
宿主
克隆类型
标记
应用范围
克隆位点
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抗原表位
- AA 1700-2300
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原理
- Recombinant Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Antibody
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特异性
- Dystrophin-glycoprotein complex (DGC) connects the F-Actin cytoskeleton on the inner surface of muscle fibers to the surrounding extracellular matrix, through the cell membrane interface. A deficiency in this protein contributes to Duchenne (DMD) and Becker (BMD) muscular dystrophies. The human dystrophin gene measures 2.4 megabases, has more than 80 exons, produces a 14 kb mRNA and contains at least 8 independent tissue-specific promoters and 2 poly A sites. The dystrophin mRNA can undergo differential splicing and produce a range of transcripts that encode a large set of proteins. Dystrophin represents approximately 0.002 % of total striated muscle protein and localizes to triadic junctions in skeletal muscle, where it is thought to influence calcium ion homeostasis and force transmission.
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纯化方法
- 200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G.
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免疫原
- Recombinant fragment (around aa1700-2300) of human DMD (exact sequence is proprietary).
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亚型
- IgG, kappa
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应用备注
- Optimal working dilution should be determined by the investigator.
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限制
- 仅限研究用
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浓度
- 1.0 mg/mL
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缓冲液
- Prepared in 10 mM PBS
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储存液
- Azide free
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储存条件
- -20 °C,-80 °C
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储存方法
- Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C.Antibody is stable for 24 months. Non-hazardous. No MSDS required.
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- Dystrophin (DMD)
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别名
- Dystrophin
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背景
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BMD, CMD3B, Duchenne muscular dystrophy (DMD), Dystrophin, Muscular dystrophy Duchenne and Becker types
Human skeletal muscle and heart muscle tissues (IHC).
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分子量
- 427kDa
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基因ID
- 1756, 495912
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UniProt
- P11532
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途径
- Skeletal Muscle Fiber Development
抗原
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