EVC2 Antikörper (AA 101-200)

Produktnummer ABIN719231

Produktdetails anti-EVC2 Antikörper

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

Bindungsspezifität: AA 101-200

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser EVC2 Antikörper ist unkonjugiert

Applikation: 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

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human EVC2

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: 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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

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

Informationen zur Lagerung: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Haltbarkeit: 12 months

Details zu EVC2

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

Andere Bezeichnung: EVC2 (EVC2 Produkte)

Synonyme: EVC2 antikoerper, LBN antikoerper, 1110017L09Rik antikoerper, Lbn antikoerper, limbin antikoerper, EvC ciliary complex subunit 2 antikoerper, EVC2 antikoerper, Evc2 antikoerper

Hintergrund:

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.

Gen-ID: 132884

Pathways: Hedgehog Signalweg