Dystrophin Antikörper (AA 114-263)
Kurzübersicht für Dystrophin Antikörper (AA 114-263) (ABIN6937092)
Target
Alle Dystrophin (DMD) Antikörper anzeigenReaktivität
Wirt
Klonalität
Konjugat
Applikation
Klon
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Bindungsspezifität
- AA 114-263
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Spezifität
- 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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Kreuzreaktivität (Details)
- Human.
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Aufreinigung
- 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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Isotyp
- IgG2b kappa
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Applikationshinweise
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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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Beschränkungen
- Nur für Forschungszwecke einsetzbar
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Konzentration
- 1.0 mg/mL
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Buffer
- Prepared in 10 mM PBS, WITHOUT BSA and Azide.
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Konservierungsmittel
- Azide free
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Lagerung
- -20 °C,-80 °C
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Informationen zur Lagerung
- Antibody without azide store at -20 to -80 °C. Antibody is stable for 24 months. Non-hazardous.
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Haltbarkeit
- 24 months
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- Dystrophin (DMD)
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Andere Bezeichnung
- DMD
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Hintergrund
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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 -
Molekulargewicht
- 427kDa
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Gen-ID
- 1756, 495912
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UniProt
- P11532
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Pathways
- Skeletal Muscle Fiber Development
Target
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