| Overview |
| bs-6638R |
| EVC2 Polyclonal Antibody |
| WB, ELISA, IHC-P, IHC-F, IF(IHC-P), IF(IHC-F), IF(ICC), ICC |
| Human |
| Specifications |
| Unconjugated |
| Rabbit |
| KLH conjugated synthetic peptide derived from human EVC2 |
| 101-200/1308 |
| Polyclonal |
| IgG |
| 1ug/ul |
| Purified by Protein A. |
| 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| Target |
| 132884 |
| Cytoplasm, Nucleus, Cell membrane |
| Ellis van Creveld syndrome 2; LBN; Limbin; LBN_HUMAN. |
| EVC2 is an integral membrane protein that plays a vital role in bone formation and skeletal development. Defects in EVC2 are a cause of Ellis-van Creveld syndrome (EVC), also known as chondroectodermal dysplasia. EVC is an autosomal recessive disorder 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. |
| Application Dilution |
| WB |
1:300-5000 |
| ELISA |
1:500-1000 |
| IHC-P |
1:200-400 |
| IHC-F |
1:100-500 |
| IF(IHC-P) |
1:50-200 |
| IF(IHC-F) |
1:50-200 |
| IF(ICC) |
1:50-200 |
| ICC |
1:100-500 |
Powered by Bioz