Dystrophin 抗体 (AA 114-263) (CF®568)
Quick Overview for Dystrophin 抗体 (AA 114-263) (CF®568) (ABIN7653323)
抗原
See all Dystrophin (DMD) 抗体适用
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 114-263
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原理
 - Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy)(DMD/3245), CF568 conjugate
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产品特性
 - 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. Primary antibodies are available purified, or with a selection of fluorescent CF® Dyes and other labels. CF® Dyes offer exceptional brightness and photostability. Note: Conjugates of blue fluorescent dyes like CF®405S and CF®405M are not recommended for detecting low abundance targets, because blue dyes have lower fluorescence and can give higher non-specific background than other dye colors.
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免疫原
 - A recombinant fragment (around aa 114-263) of human DMD protein
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亚型
 - IgG2a, kappa
 
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应用备注
 - Optimal working dilution should be determined by the investigator.
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说明
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Positive Control: Human skeletal muscle and heart muscle tissues (IHC).
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限制
 - 仅限研究用
 
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状态
 - Liquid
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浓度
 - 0.1 mg/mL
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缓冲液
 - PBS, 0.1 % BSA, 0.05 % azide
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储存液
 - Sodium azide
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注意事项
 - This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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注意事项
 - Protect from light
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储存条件
 - 4 °C
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储存方法
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Stable at room temperature or 37°C for 7 days.
Protect from light
Store at 2 to 8°C. Protect fluorescent conjugates from light
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有效期
 - 24 months
 
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- Dystrophin (DMD)
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别名
 - Dystrophin
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背景
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Synonyms: BMD, CMD3B, Duchenne muscular dystrophy (DMD), Dystrophin, Muscular dystrophy Duchenne and Becker types
Gene Symbol: DMD
Tissue Expression: Muscle
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分子量
 - 427 kDa
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基因ID
 - 1756
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UniProt
 - P11532
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途径
 - Skeletal Muscle Fiber Development
 
抗原
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