Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002570027 | SCV002934578 | pathogenic | Developmental and epileptic encephalopathy, 1; Autosomal recessive spinocerebellar ataxia 12 | 2022-11-07 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. This variant disrupts a region of the WWOX protein in which other variant(s) (p.Pro47Thr) have been determined to be pathogenic (PMID: 24369382). This suggests that this is a clinically significant region of the protein, and that variants that disrupt it are likely to be disease-causing. This variant has not been reported in the literature in individuals affected with WWOX-related conditions. This variant is present in population databases (rs758588684, gnomAD 0.03%). This sequence change affects the initiator methionine of the WWOX mRNA. The next in-frame methionine is located at codon 114. |