Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Counsyl | RCV000667482 | SCV000791937 | likely pathogenic | Ellis-van Creveld syndrome | 2017-06-21 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV003767956 | SCV004573494 | pathogenic | Ellis-van Creveld syndrome; Curry-Hall syndrome | 2023-03-31 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 552255). This variant has not been reported in the literature in individuals affected with EVC-related conditions. The frequency data for this variant in the population databases is considered unreliable, as metrics indicate insufficient coverage at this position in the gnomAD database. This sequence change creates a premature translational stop signal (p.Cys8*) in the EVC gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in EVC are known to be pathogenic (PMID: 23220543). |