Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ce |
RCV000997258 | SCV001152480 | likely pathogenic | not provided | 2019-01-01 | criteria provided, single submitter | clinical testing | |
Pediatrics, |
RCV001263485 | SCV001441531 | likely pathogenic | Developmental and epileptic encephalopathy, 11 | criteria provided, single submitter | clinical testing | This variant is absent in population databases. This variant has not been described in the literature, but is submitted in ClinVar as a likely pathogenic. Parents were also tested and this variant was not detected. Algorithms developed to predict the effect of missense variants showed: Sift- Deleterious, PolyPhen2-HDIV- Probably damaging, MutationTaster- Damaging, Provean - Damaging. This alanine residue is highly conserved. | |
Labcorp Genetics |
RCV001324429 | SCV001515381 | likely pathogenic | Seizures, benign familial infantile, 3; Developmental and epileptic encephalopathy, 11 | 2021-01-05 | criteria provided, single submitter | clinical testing | In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. This sequence change replaces alanine with serine at codon 880 of the SCN2A protein (p.Ala880Ser). The alanine residue is highly conserved and there is a moderate physicochemical difference between alanine and serine. This variant is not present in population databases (ExAC no frequency). This variant has been observed in individual(s) with clinical features of SCN2A-related conditions (Invitae). In at least one individual the variant was observed to be de novo. ClinVar contains an entry for this variant (Variation ID: 808846). 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"). |
Genome |
RCV001265497 | SCV001443641 | pathogenic | Complex neurodevelopmental disorder | 2016-09-15 | no assertion criteria provided | provider interpretation | Submission from Simons Searchlight facilitated by GenomeConnect. Variant interpreted by the Simons Searchlight team most recently on 2016-09-15 and interpreted as Pathogenic. Variant was initially reported on 2015-04-10 by GTR ID of laboratory name North East Thames Genetic Service. The reporting laboratory might also submit to ClinVar. |
Channelopathy- |
RCV001265497 | SCV004232420 | not provided | Complex neurodevelopmental disorder | no assertion provided | literature only |