Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000696324 | SCV000824879 | uncertain significance | Early infantile epileptic encephalopathy with suppression bursts | 2018-05-02 | 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 do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Possibly Damaging"; Align-GVGD: "Class C0"). This variant has not been reported in the literature in individuals with SCN8A-related disease. This variant is not present in population databases (ExAC no frequency). This sequence change replaces alanine with valine at codon 982 of the SCN8A protein (p.Ala982Val). The alanine residue is highly conserved and there is a small physicochemical difference between alanine and valine. |
Institute of Human Genetics, |
RCV002255511 | SCV002526712 | likely pathogenic | Seizures, benign familial infantile, 5 | 2022-05-24 | criteria provided, single submitter | clinical testing | This variant was identified as de novo (maternity and paternity confirmed)._x000D_ Criteria applied: PS2_MOD, PM1, PM2_SUP, PP3 |