Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV005115003 | SCV005736517 | pathogenic | Early infantile epileptic encephalopathy with suppression bursts | 2024-10-17 | criteria provided, single submitter | clinical testing | This sequence change replaces tyrosine, which is neutral and polar, with aspartic acid, which is acidic and polar, at codon 168 of the GNAO1 protein (p.Tyr168Asp). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individual(s) with GNAO1-related conditions (internal data). In at least one individual the variant was observed to be de novo. 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 expected to disrupt GNAO1 protein function with a positive predictive value of 95%. For these reasons, this variant has been classified as Pathogenic. |