Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV002254821 | SCV002525914 | pathogenic | Glanzmann thrombasthenia | 2022-04-15 | reviewed by expert panel | curation | The NM_000419.5(ITGA2B):c.2578C>T (p.Gln860Ter) nonsense variant in exon 25/30 is predicted to lead to nonsense mediated decay in a gene in which loss-of-function is an established disease mechanism (PVS1). Glanzmann thrombasthenia Patient 6 of PMID: 34267460 is homozygous for this variant (PM3_supporting). This variant is absent from gnomAD v2.1.1 (PM2_Supporting). In summary this variant meets criteria to be classified as Pathogenic for autosomal recessive Glanzmann Thrombasthenia based on the ACMG/AMP criteria applied, as specified by the ClinGen PD VCEP: PVS1, PM2_supporting, PM3_supporting. (VCEP specifications version 2.1) |