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EVC Antikörper (AA 251-350) (AbBy Fluor® 488)

EVC Reaktivität: Human IF (cc), IF (p) Wirt: Kaninchen Polyclonal AbBy Fluor® 488
Produktnummer ABIN1390342
  • Target Alle EVC Antikörper anzeigen
    EVC (Ellis Van Creveld Syndrome (EVC))
    Bindungsspezifität
    • 14
    • 7
    • 2
    • 1
    AA 251-350
    Reaktivität
    • 24
    • 1
    Human
    Wirt
    • 20
    • 4
    Kaninchen
    Klonalität
    • 23
    • 1
    Polyklonal
    Konjugat
    • 7
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Dieser EVC Antikörper ist konjugiert mit AbBy Fluor® 488
    Applikation
    • 12
    • 12
    • 9
    • 6
    • 3
    • 3
    • 1
    • 1
    Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    Homologie
    Human
    Aufreinigung
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human EVC1
    Isotyp
    IgG
    Top Product
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  • Applikationshinweise
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    Beschränkungen
    Nur für Forschungszwecke einsetzbar
  • Format
    Liquid
    Konzentration
    1 μg/μL
    Buffer
    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
    Konservierungsmittel
    ProClin
    Vorsichtsmaßnahmen
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Lagerung
    -20 °C
    Informationen zur Lagerung
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Haltbarkeit
    12 months
  • Target
    EVC (Ellis Van Creveld Syndrome (EVC))
    Andere Bezeichnung
    EVC1 (EVC Produkte)
    Synonyme
    EVC antikoerper, DWF-1 antikoerper, evcl antikoerper, dwf-1 antikoerper, EVC1 antikoerper, EVCL antikoerper, EvC ciliary complex subunit 1 antikoerper, EVC antikoerper, evc antikoerper, Evc antikoerper
    Hintergrund

    Synonyms: Ellis van Creveld syndrome DWF 1, DWF1, Ellis van Creveld syndrome protein, Ellis-van Creveld syndrome, EVC, EVC1, EVC 1, EVC-1, EVCL, MGC105107, EVC_HUMAN.

    Background: EVC is an autosomal skeletal dysplasia caused by mutations in the EVC and EVC2 genes. Found in developing ribs, heart, kidney and lung, the EVC gene is responsible for normal development of the face, limbs, teeth and nails. The protein expressed by the EVC gene is an intracellular component of the hedgehog signal pathway that contains a leucine zipper and transmembrane domain. Defects in the EVC gene can lead to short-limb dwarfism, ectodermal dysplasia and cardiac anomalies such as irregular atrioventricular septum development. Additionally, the EVC gene has been implicated in Weyers acrodental dysostosis, an autosomal dominant disease characterized by facial abnormalities and limb defects.

    Pathways
    Hedgehog Signalweg
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