Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001211933 | SCV001383501 | uncertain significance | Early infantile epileptic encephalopathy with suppression bursts | 2019-09-01 | criteria provided, single submitter | clinical testing | 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. Algorithms developed to predict the effect of missense changes on protein structure and function are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Probably Damaging"; Align-GVGD: "Class C0"). This variant has not been reported in the literature in individuals with SCN8A-related conditions. This variant is not present in population databases (ExAC no frequency). This sequence change replaces valine with methionine at codon 1195 of the SCN8A protein (p.Val1195Met). The valine residue is highly conserved and there is a small physicochemical difference between valine and methionine. |
Institute of Human Genetics, |
RCV002468624 | SCV002765120 | uncertain significance | Seizures, benign familial infantile, 5 | 2022-12-07 | criteria provided, single submitter | clinical testing | _x000D_This variant was identified as compound heterozygous with NM_014191.4:c.2702A>G. Criteria applied: PM2_SUP, PP3, PP2 |
Institute of Human Genetics, |
RCV003128380 | SCV003804684 | uncertain significance | Developmental and epileptic encephalopathy, 13 | 2023-01-04 | criteria provided, single submitter | clinical testing | This variant was identified as compound heterozygous with NM_014191.4:c.2702A>G, p.(Tyr901Cys). Criteria applied: PM2_SUP, PP3, PP2 |