Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratorio de Genetica e Diagnostico Molecular, |
RCV002252387 | SCV002523506 | uncertain significance | See cases | 2020-02-10 | criteria provided, single submitter | clinical testing | ACMG classification criteria: PM2, BP4 |
Labcorp Genetics |
RCV003101382 | SCV002931036 | uncertain significance | not provided | 2024-10-18 | criteria provided, single submitter | clinical testing | This sequence change replaces tyrosine, which is neutral and polar, with histidine, which is basic and polar, at codon 2641 of the PIEZO2 protein (p.Tyr2641His). This variant is present in population databases (rs753719142, gnomAD 0.003%). This variant has not been reported in the literature in individuals affected with PIEZO2-related conditions. ClinVar contains an entry for this variant (Variation ID: 1690795). Invitae Evidence Modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) indicates that this missense variant is not expected to disrupt PIEZO2 protein function with a negative predictive value of 95%. 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. |