Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000011780 | SCV000754741 | pathogenic | Hypohidrotic X-linked ectodermal dysplasia | 2017-11-02 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Gln23*) in the EDA gene. It is expected to result in an absent or disrupted protein product. For these reasons, this variant has been classified as Pathogenic. Loss-of-function variants in EDA are known to be pathogenic (PMID: 9683615). This variant has been reported in the literature in an individual affected with X linked hypohidrotic ectodermal dysplasia (PMID: 9507389). ClinVar contains an entry for this variant (Variation ID: 11033). This variant is not present in population databases (ExAC no frequency). |
Gene |
RCV004786252 | SCV005401387 | pathogenic | not provided | 2024-05-14 | criteria provided, single submitter | clinical testing | Nonsense variant predicted to result in protein truncation or nonsense mediated decay in a gene for which loss of function is a known mechanism of disease; Not observed at significant frequency in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 9507389) |
OMIM | RCV000011780 | SCV000032012 | pathogenic | Hypohidrotic X-linked ectodermal dysplasia | 1998-02-01 | no assertion criteria provided | literature only |