Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV000852029 | SCV001809902 | uncertain significance | Glanzmann thrombasthenia | 2024-06-06 | reviewed by expert panel | curation | The NM_000419.5(ITGA2B):c.1440-1G>A splice variant is predicted to result in the loss of exon 15 causing an in-frame deletion of 35 amino acids. This occurs in the the critical ligand binding extracellular domain (PVS1_Strong). This variant is absent from gnomAD v4.0 (PM2_Supporting). It reported in one patient stated to have a disease of platelet function (PMID: 31064749) but information was insufficient or inconsistent to apply any evidence for this patient. In summary, based on the evidence available at this time, the variant is classified as uncertain significance. GT-specific criteria applied: PVS1_strong, PM2_supporting. |
NIHR Bioresource Rare Diseases, |
RCV000852029 | SCV000899505 | pathogenic | Glanzmann thrombasthenia | 2019-02-01 | criteria provided, single submitter | research |