EVC antibody (AA 251-350) (AbBy Fluor® 488)

Catalog No. ABIN1390342

Product Details anti-EVC Antibody (AbBy Fluor® 488)

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

Binding Specificity: AA 251-350

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This EVC antibody is conjugated to AbBy Fluor® 488

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

Predicted Reactivity: Human

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human EVC1

Isotype: IgG

Application Details

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

Restrictions: For Research Use only

Handling

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.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for EVC

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

Alternative Name: EVC1 (EVC Products)

Synonyms: EVC antibody, DWF-1 antibody, evcl antibody, dwf-1 antibody, EVC1 antibody, EVCL antibody, EvC ciliary complex subunit 1 antibody, EVC antibody, evc antibody, Evc antibody

Background:

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 Signaling