Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001855860 | SCV002278677 | uncertain significance | Congenital primary aphakia; Anterior segment dysgenesis | 2024-04-15 | criteria provided, single submitter | clinical testing | This sequence change replaces proline, which is neutral and non-polar, with leucine, which is neutral and non-polar, at codon 202 of the FOXE3 protein (p.Pro202Leu). This variant is present in population databases (rs745950487, gnomAD 0.1%). This missense change has been observed in individual(s) with familial thoracic aortic aneurysm and/or dissection (PMID: 26854927). ClinVar contains an entry for this variant (Variation ID: 617854). Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is not expected to disrupt FOXE3 protein function with a negative predictive value of 80%. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
University of Washington Center for Mendelian Genomics, |
RCV000755154 | SCV000882976 | likely benign | Congenital aneurysm of ascending aorta; Acute aortic dissection | 2016-02-08 | no assertion criteria provided | research |