Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001803440 | SCV002047592 | pathogenic | Glanzmann thrombasthenia | 2021-09-03 | reviewed by expert panel | curation | NM_000419.5(ITGA2B):c.2421G>A (p.Trp807Ter) is a nonsense variant located on exon 24 which is predicted to result in NMD. It is absent from all large population cohorts (gnomAD). This variant has been reported to occur in homozygous state in one proband who meets the diagnostic criteria for the GT phenotype (PMID: 25728920). This variant meets GT specific criteria for PVS1, PP4_moderate, PM2_supporting, and PM3_supporting and is therefore classified as Pathogenic. |