EVC2 anticorps (AA 101-200)

N° du produit ABIN719231

Détail du produit anti-EVC2 anticorps

Antigène: EVC2 (Ellis Van Creveld Syndrome 2 (EVC2))

Épitope: AA 101-200

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp EVC2 est non-conjugé

Application: ELISA, Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunocytochemistry (ICC), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Homologie: Human

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human EVC2

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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: 4 °C,-20 °C

Stockage commentaire: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Date de péremption: 12 months

Détails sur EVC2

Antigène: EVC2 (Ellis Van Creveld Syndrome 2 (EVC2))

Autre désignation: EVC2 (EVC2 Produits)

Synonymes: anticorps EVC2, anticorps LBN, anticorps 1110017L09Rik, anticorps Lbn, anticorps limbin, anticorps EvC ciliary complex subunit 2, anticorps EVC2, anticorps Evc2

Sujet:

Synonyms: Ellis van Creveld syndrome 2, LBN, Limbin, LBN_HUMAN.

Background: EVC2 is an integral membrane protein that plays a vital role in bone formation and skeletal development. Defects in EVC2 are a cause of Ellis-van Creveld syndrome (EVC), also known as chondroectodermal dysplasia. EVC is an autosomal recessive disorder characterized by the clinical tetrad of chondrodystrophy, polydactyly, ectodermal dysplasia and cardiac anomalies. Patients manifest short-limb dwarfism, short ribs, postaxial polydactyly and dysplastic nails and teeth. Congenital heart defects, most commonly an atrioventricular septal defect, are observed in 60 % of affected individuals.

ID gène: 132884

Pathways: Signalisation Hedgehog