Dystrophin 抗体 (AA 114-263)
Quick Overview for Dystrophin 抗体 (AA 114-263) (ABIN6937095)
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
-
-
抗原表位
- AA 114-263
-
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.
-
交叉反应 (详细)
- Human.
-
纯化方法
- 1.0mg/ml of Ab purified from Bioreactor by Protein A/G.
-
免疫原
- A recombinant fragment (around aa 114-263) of human DMD protein (exact sequence is proprietary)
-
亚型
- IgG2a kappa
-
-
-
-
应用备注
-
Known_Application: ELISA (For coating, order Ab without BSA),Immunohistochemistry (Formalin-fixed) (1-2 μg/mL for 30 minutes at RT),(Staining of formalin-fixed tissues requires heating tissue sections in 10 mM Tris with 1 mM EDTA, pH 9.0, for 45 min at 95°C followed by cooling at RT for 20 minutes), Optimal dilution for a specific application should be determined.
Positive_Control: Human skeletal muscle and heart muscle tissues (IHC).
-
限制
- 仅限研究用
-
-
-
浓度
- 1.0 mg/mL
-
缓冲液
- Prepared in 10 mM PBS, WITHOUT BSA and Azide.
-
储存液
- Azide free
-
储存条件
- -20 °C,-80 °C
-
储存方法
- Antibody without azide store at -20 to -80 °C. Antibody is stable for 24 months. Non-hazardous.
-
有效期
- 24 months
-
-
- Dystrophin (DMD)
-
别名
- DMD
-
背景
-
BMD, CMD3B, Duchenne muscular dystrophy (DMD), Dystrophin, Muscular dystrophy Duchenne and Becker types,Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy)
Cellular localisation: Cell Surface and Cytoplasmic -
分子量
- 427kDa
-
基因ID
- 1756, 495912
-
UniProt
- P11532
-
途径
- Skeletal Muscle Fiber Development
抗原
-