Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV002264785 | SCV002546441 | likely pathogenic | Hereditary thrombocytopenia and hematologic cancer predisposition syndrome | 2022-03-21 | reviewed by expert panel | curation | NM_001754.5(RUNX1):c.253_254insGGGG (p.His85fs) is a frameshift variant predicted to undergo NMD (PVS1, SNV tree). This variant is completely absent from all population databases with at least 20x coverage for RUNX1 (PM2_supporting). In summary, this variant meets criteria to be classified as likely pathogenic. ACMG/AMP criteria applied, as specified by the Myeloid Malignancy Variant Curation Expert Panel for RUNX1: PVS1 and PM2_supporting |
Labcorp Genetics |
RCV001386548 | SCV001586809 | pathogenic | Hereditary thrombocytopenia and hematological cancer predisposition syndrome associated with RUNX1 | 2020-02-19 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Loss-of-function variants in RUNX1 are known to be pathogenic (PMID: 18723428, 24100448). This variant has not been reported in the literature in individuals with RUNX1-related conditions. This variant is not present in population databases (ExAC no frequency). This sequence change creates a premature translational stop signal (p.His85Argfs*54) in the RUNX1 gene. It is expected to result in an absent or disrupted protein product. |