Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003603997 | SCV004552376 | pathogenic | Pyruvate dehydrogenase E2 deficiency | 2023-12-31 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Gln190*) in the DLAT gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in DLAT are known to be pathogenic (PMID: 20022530, 23021068). This variant is present in population databases (rs782225633, gnomAD 0.03%). This variant has not been reported in the literature in individuals affected with DLAT-related conditions. For these reasons, this variant has been classified as Pathogenic. |