Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Pediatrics Genetics, |
RCV003129592 | SCV003804995 | likely pathogenic | Lafora disease | 2023-03-01 | criteria provided, single submitter | clinical testing | PVS1, PM2 |
Labcorp Genetics |
RCV003746657 | SCV004377227 | pathogenic | Progressive myoclonic epilepsy | 2023-07-29 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 2431059). This premature translational stop signal has been observed in individual(s) with Lafora disease (PMID: 10932264). This variant is not present in population databases (gnomAD no frequency). This sequence change creates a premature translational stop signal (p.Trp60*) in the EPM2A gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in EPM2A are known to be pathogenic (PMID: 20738377). |