Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003055458 | SCV003340677 | pathogenic | Mitochondrial DNA depletion syndrome, encephalomyopathic form with methylmalonic aciduria | 2023-08-04 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. This sequence change creates a premature translational stop signal (p.Tyr7*) in the SUCLA2 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in SUCLA2 are known to be pathogenic (PMID: 15877282, 17301081). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with SUCLA2-related conditions. ClinVar contains an entry for this variant (Variation ID: 2127356). |