Dystrophin antibody (AA 114-263)
Quick Overview for Dystrophin antibody (AA 114-263) (ABIN6937095)
Target
See all Dystrophin (DMD) AntibodiesReactivity
Host
Clonality
Conjugate
Application
Clone
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Binding Specificity
- AA 114-263
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Specificity
- 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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Cross-Reactivity (Details)
- Human.
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Purification
- 1.0mg/ml of Ab purified from Bioreactor by Protein A/G.
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Immunogen
- A recombinant fragment (around aa 114-263) of human DMD protein (exact sequence is proprietary)
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Isotype
- IgG2a kappa
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Application Notes
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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).
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Restrictions
- For Research Use only
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Concentration
- 1.0 mg/mL
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Buffer
- Prepared in 10 mM PBS, WITHOUT BSA and Azide.
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Preservative
- Azide free
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Storage
- -20 °C,-80 °C
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Storage Comment
- Antibody without azide store at -20 to -80 °C. Antibody is stable for 24 months. Non-hazardous.
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Expiry Date
- 24 months
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- Dystrophin (DMD)
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Alternative Name
- DMD
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Background
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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 -
Molecular Weight
- 427kDa
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Gene ID
- 1756, 495912
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UniProt
- P11532
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Pathways
- Skeletal Muscle Fiber Development
Target
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