EVC Antikörper (AA 251-350) (Biotin)

Produktnummer ABIN1390345

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch EVC in ELISA, IHC (fro) und IHC (p). Er zeigt eine Reaktivität gegenüber Human.

Kurzübersicht für EVC Antikörper (AA 251-350) (Biotin) (ABIN1390345)

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser EVC Antikörper ist konjugiert mit Biotin

Applikation: ELISA, Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Produktdetails anti-EVC Antikörper (Biotin)

Bindungsspezifität: AA 251-350

Homologie: Human

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human EVC1

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IHC-P 1:200-400
IHC-F 1:100-500

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 for 12 months.

Haltbarkeit: 12 months

Details zu EVC

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

Andere Bezeichnung: EVC1

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