Validation Data Gallery
Tested Applications
Recommended dilution
| Application | Dilution |
|---|---|
| It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
Product Information
55367-1-PBS targets EVC2 in WB, IHC, Indirect ELISA applications and shows reactivity with human, mouse samples.
| Tested Reactivity | human, mouse |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | Peptide 相同性解析による交差性が予測される生物種 |
| Full Name | Ellis van Creveld syndrome 2 |
| Calculated molecular weight | 148 kDa |
| Observed molecular weight | 140 kDa |
| GenBank accession number | NM_147127 |
| Gene Symbol | EVC2 |
| Gene ID (NCBI) | 132884 |
| Conjugate | Unconjugated |
| Form | Liquid |
| Purification Method | Antigen affinity purification |
| UNIPROT ID | Q86UK5 |
| Storage Buffer | PBS only{{ptg:BufferTemp}}7.3 |
| Storage Conditions | Store at -80°C. |
Background Information
EVC2 (also known as LIMBIN) is close proximity in divergent orientation to EVC and mutations in both human genes lead to the chondrodysplasia Ellis-van Creveld (EvC) syndrome. Typical symptoms of EvC include shorter limbs and ribs, postaxial polydactyly, dysplastic nails, and cardiovascular defects (PMID: 28081373). Additionally, EvC syndrome has been characterized as a ciliopathy because the EVC and EVC2 proteins are intracellularly localized at the bottom of the primary cilium, where they form a heterotrimeric protein complex with SMO and transduce Hedgehog(Hh) signaling (PMID: 30410447). EVC2 is expressed in the heart, placenta, lung, liver, skeletal muscle, kidney, and pancreas. EVC2 has 3 isoforms with the molecular mass of 140-148 kDa.