Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Revvity Omics, |
RCV001783467 | SCV002023129 | pathogenic | Cranioectodermal dysplasia 1 | 2020-03-13 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001783467 | SCV003255668 | pathogenic | Cranioectodermal dysplasia 1 | 2024-11-27 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Ser1194Argfs*3) in the IFT122 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in IFT122 are known to be pathogenic (PMID: 20493458, 23826986, 26792575). This variant is present in population databases (no rsID available, gnomAD 0.003%). This variant has not been reported in the literature in individuals affected with IFT122-related conditions. ClinVar contains an entry for this variant (Variation ID: 1323103). For these reasons, this variant has been classified as Pathogenic. |