bs-11282R-BF350

DATASHEET

Host: Rabbit

Target Protein: EVC1

Immunogen Range: 251-350/408

Clonality: Polyclonal

Isotype: IgG

Source: KLH conjugated synthetic peptide derived from human EVC1

Purification: Purified by Protein A.

Storage Buffer: Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

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

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.

Conjugation: AbBy Fluor® 350

Excitation/ Emission: 346nm/442nm

Size: 100ul

Concentration: 1ug/ul

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

Predicted Molecular Weight: 112

Predicted Cross Reactive Species: Human

For research use only. Not intended for diagnostic or therapeutic use.