Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000690341 | SCV000818023 | uncertain significance | Developmental and epileptic encephalopathy, 1; Autosomal recessive spinocerebellar ataxia 12 | 2018-01-08 | criteria provided, single submitter | clinical testing | This sequence change replaces threonine with arginine at codon 12 of the WWOX protein (p.Thr12Arg). The threonine residue is moderately conserved and there is a moderate physicochemical difference between threonine and arginine. 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 WWOX-related disease. This variant is not present in population databases (ExAC no frequency). |
Equipe Genetique des Anomalies du Developpement, |
RCV002286420 | SCV002576487 | pathogenic | Autosomal recessive spinocerebellar ataxia 12 | criteria provided, single submitter | clinical testing |