EVC1 Polyclonal Antibody, Biotin Conjugated
Product Specifications
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.
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.
Cellular Locus
Cell membrane
Host
Rabbit
Immunogen
251-350/408
Target
EVC1
Clonality
Polyclonal
Isotype
IgG
Conjugation
Biotin
Source
KLH conjugated synthetic peptide derived from human EVC1
Applications
ELISA, IHC-P, IHC-F
Purification
Purified by Protein A.
Concentration
1µg/µl
Dilution
ELISA (1:500-1000), IHC-P (1:200-400), IHC-F (1:100-500)
Buffer
Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Store at -20°C for 12 months.
Predicted Cross Reactivity
Human
Curated Selection
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