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EVC anticorps (AA 251-350) (Cy5.5)

EVC Reactivité: Humain IF (cc), IF (p) Hôte: Lapin Polyclonal Cy5.5
N° du produit ABIN1408235
  • Antigène Voir toutes EVC Anticorps
    EVC (Ellis Van Creveld Syndrome (EVC))
    Épitope
    • 14
    • 7
    • 2
    • 1
    AA 251-350
    Reactivité
    • 24
    • 2
    Humain
    Hôte
    • 21
    • 4
    Lapin
    Clonalité
    • 24
    • 1
    Polyclonal
    Conjugué
    • 8
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Cet anticorp EVC est conjugé à/à la Cy5.5
    Application
    • 12
    • 12
    • 9
    • 7
    • 3
    • 3
    • 1
    • 1
    Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    Homologie
    Human
    Purification
    Purified by Protein A.
    Immunogène
    KLH conjugated synthetic peptide derived from human EVC1
    Isotype
    IgG
    Top Product
    Discover our top product EVC Anticorps primaire
  • Indications d'application
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    Restrictions
    For Research Use only
  • Format
    Liquid
    Concentration
    1 μg/μL
    Buffer
    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
    Agent conservateur
    ProClin
    Précaution d'utilisation
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Stock
    -20 °C
    Stockage commentaire
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Date de péremption
    12 months
  • Antigène
    EVC (Ellis Van Creveld Syndrome (EVC))
    Autre désignation
    EVC1 (EVC Produits)
    Synonymes
    anticorps EVC, anticorps DWF-1, anticorps evcl, anticorps dwf-1, anticorps EVC1, anticorps EVCL, anticorps EvC ciliary complex subunit 1, anticorps EVC, anticorps evc, anticorps Evc
    Sujet

    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
    Signalisation Hedgehog
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