产品详情
简单介绍：
Anti-EVC2 Antibody
详情介绍：
Overview
| Name: | Anti-EVC2 Antibody See all EVC2 primary antibodies
|
| Description: | Rabbit polyclonal antibody to EVC2 |
| Specificity: | The antibody detects endogenous levels of total EVC2 protein. |
| Applications: | WB, IHC |
| Reactivity: | Human |
| Immunogen: | Synthetic peptide corresponding to a region derived from internal residues of human Ellis van Creveld syndrome 2 |
| Host: | Rabbit |
| Clonality: | Polyclonal |
| Conjugate: | Unconjugated |
| Purification: | Antigen affinity purification. |
| Concentration: | 2.6mg / ml |
| Formulation: | Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol. |
| Storage: | Store at -20?C |
Target
| Function: | Positive regulator of the hedgehog signaling pathway (By similarity). Plays a critical role in bone formation and skeletal development. |
| Tissue Specificity: | Found in the heart, placenta, lung, liver, skeletal muscle, kidney and pancreas. |
| Involvement in Disease: | Ellis-van Creveld syndrome: An autosomal recessive condition characterized by the clinical tetrad of chondrodystrophy, polydactyly, ectodermal dysplasia and cardiac anomalies. Patients manifest short-limb dwarfism, short ribs, postaxial polydactyly, and dysplastic nails and teeth. Congenital heart defects, most commonly an atrioventricular septal defect, are observed in 60% of affected individuals. Acrofacial dysostosis, Weyers type: An autosomal dominant condition characterized by dysplastic nails, postaxial polydactyly, dental anomalies, short limbs, short stature and normal intelligence. The phenotype is milder than Ellis-van Creveld syndrome. |
| Cellular Location: | Cell membrane. Cytoplasm > Cytoskeleton > Cilium basal body. Cell projection > Cilium. Cell projection > Cilium membrane. Nucleus. |
| Database Links: |
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| Synonyms: | |
| Information: | Target information shown above is from the UniProt Consortium. |
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