Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Revvity Omics, |
RCV003137066 | SCV003825436 | pathogenic | not provided | 2022-03-04 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV003137066 | SCV005834758 | uncertain significance | not provided | 2024-08-16 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg559*) in the PKLR gene. While this is not anticipated to result in nonsense mediated decay, it is expected to disrupt the last 16 amino acid(s) of the PKLR protein. This variant is present in population databases (rs532230312, gnomAD 0.01%). This premature translational stop signal has been observed in individual(s) with pyruvate kinase deficiency (PMID: 9827908, 34201899, 36892591). ClinVar contains an entry for this variant (Variation ID: 2437900). 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. |