Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000638913 | SCV000760468 | uncertain significance | Familial meningioma | 2023-09-21 | criteria provided, single submitter | clinical testing | This variant has not been reported in the literature in individuals affected with SMARCE1-related conditions. This variant is present in population databases (rs768536854, gnomAD 0.003%). This sequence change replaces alanine, which is neutral and non-polar, with valine, which is neutral and non-polar, at codon 311 of the SMARCE1 protein (p.Ala311Val). ClinVar contains an entry for this variant (Variation ID: 532236). 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 SMARCE1 protein function. |
Ambry Genetics | RCV001019177 | SCV001180502 | uncertain significance | Hereditary cancer-predisposing syndrome | 2021-10-15 | criteria provided, single submitter | clinical testing | The p.A311V variant (also known as c.932C>T), located in coding exon 9 of the SMARCE1 gene, results from a C to T substitution at nucleotide position 932. The alanine at codon 311 is replaced by valine, an amino acid with similar properties. This amino acid position is highly conserved in available vertebrate species. In addition, this alteration is predicted to be tolerated by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Baylor Genetics | RCV000638913 | SCV004205032 | uncertain significance | Familial meningioma | 2023-09-25 | criteria provided, single submitter | clinical testing |