EVC Antikörper (AA 251-350) (Alexa Fluor 647)

Produktnummer ABIN1390344

Produktdetails anti-EVC Antikörper (Alexa Fluor 647)

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

Bindungsspezifität: AA 251-350

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser EVC Antikörper ist konjugiert mit Alexa Fluor 647

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

Homologie: Human

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human EVC1

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

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

Details zu EVC

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