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EVC1 Polyclonal Antibody, Biotin Conjugated

Applications

  • ELISA
  • IHC-P
  • IHC-F

Predicted Reactivity

  • Human
Overview
Catalog # bs-11282R-Biotin
Product Name EVC1 Polyclonal Antibody, Biotin Conjugated
Applications ELISA, IHC-P, IHC-F
Predicted Reactivity Human
Specifications
Conjugation Biotin
Host Rabbit
Source KLH conjugated synthetic peptide derived from human EVC1
Immunogen Range 251-350/408
Clonality Polyclonal
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Condition Store at -20°C for 12 months.
Target
Subcellular location Cell membrane
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.
Application Dilution
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500