Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001770747 | SCV001992781 | uncertain significance | not provided | 2019-06-20 | criteria provided, single submitter | clinical testing | In silico analysis, which includes protein predictors and evolutionary conservation, supports that this variant does not alter protein structure/function; Has not been previously published as pathogenic or benign to our knowledge |
Greenwood Genetic Center Diagnostic Laboratories, |
RCV001770747 | SCV002568268 | uncertain significance | not provided | 2022-04-13 | criteria provided, single submitter | clinical testing | PM2 |
Labcorp Genetics |
RCV002540283 | SCV002996991 | uncertain significance | Syndromic X-linked intellectual disability 14 | 2023-03-14 | criteria provided, single submitter | clinical testing | This sequence change replaces glutamic acid, which is acidic and polar, with lysine, which is basic and polar, at codon 328 of the UPF3B protein (p.Glu328Lys). 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. 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 UPF3B protein function. ClinVar contains an entry for this variant (Variation ID: 1306567). This variant has not been reported in the literature in individuals affected with UPF3B-related conditions. This variant is present in population databases (rs376175156, gnomAD 0.002%). |