EVC anticorps (AA 251-350) (AbBy Fluor® 350)

N° du produit ABIN1390341

Détail du produit anti-EVC anticorps (AbBy Fluor® 350)

Antigène: EVC (Ellis Van Creveld Syndrome (EVC))

Épitope: AA 251-350

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp EVC est conjugé à/à la AbBy Fluor® 350

Application: Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunofluorescence (Cultured Cells) (IF (cc))

Homologie: Human

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human EVC1

Isotype: IgG

Information d'application

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

Stockage

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

Détails sur EVC

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