Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001886138 | SCV002157305 | uncertain significance | Gastrointestinal stromal tumor | 2023-09-13 | criteria provided, single submitter | clinical testing | This variant is present in population databases (rs745658205, gnomAD 0.007%). 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 expected to disrupt PDGFRA protein function. ClinVar contains an entry for this variant (Variation ID: 1379958). This variant has not been reported in the literature in individuals affected with PDGFRA-related conditions. This sequence change replaces histidine, which is basic and polar, with glutamine, which is neutral and polar, at codon 654 of the PDGFRA protein (p.His654Gln). |
Ambry Genetics | RCV002422955 | SCV002720144 | uncertain significance | Hereditary cancer-predisposing syndrome | 2024-08-12 | criteria provided, single submitter | clinical testing | The p.H654Q variant (also known as c.1962T>A), located in coding exon 13 of the PDGFRA gene, results from a T to A substitution at nucleotide position 1962. The histidine at codon 654 is replaced by glutamine, an amino acid with highly similar properties. This amino acid position is conserved. In addition, this alteration is predicted to be deleterious by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Baylor Genetics | RCV003475140 | SCV004200949 | uncertain significance | Polyps, multiple and recurrent inflammatory fibroid, gastrointestinal | 2023-07-15 | criteria provided, single submitter | clinical testing |