Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV003448344 | SCV004176272 | pathogenic | Hereditary thrombocytopenia and hematologic cancer predisposition syndrome | 2023-12-09 | reviewed by expert panel | curation | NM_001754.5(RUNX1):c.861C>G (p.Tyr287Ter) is a nonsense variant which affects all biologically relevant isoforms. This variant is a nonsense variant between c.98 - c.916, ranking at PVS1 according to the RUNX1 decision tree (PVS1). This variant was found to co-segregate with disease in 3 affected family members (PMID: 11830488, Pedigree 3) (PP1). This variant is completely absent from all population databases with at least 20x coverage for RUNX1 (PM2_supporting). This variant is a nonsense/frameshift variants that is downstream of c.98 (PM5_Supporting). In summary, this variant meets criteria to be classified as pathogenic. ACMG/AMP criteria applied, as specified by the Myeloid Malignancy Variant Curation Expert Panel for RUNX1: PVS1, PP1, PM2_supporting, PM5_supporting. |
NIHR Bioresource Rare Diseases, |
RCV000851904 | SCV000899969 | likely pathogenic | Thrombocytopenia | 2019-02-01 | criteria provided, single submitter | research |