Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001225282 | SCV001397552 | pathogenic | Glanzmann thrombasthenia | 2020-09-04 | reviewed by expert panel | curation | The NM_000419.4:c.2915dup variant in exon 28 causes a frameshift and results in premature termination in exon 30, Leu973AlafsTer63. In-vitro studies show absent integrin expression on the cell surface (PMID: 15748238). At least 5 individuals including a sibling pair with this variant have been reported in the literature. In summary, based on the evidence available at this time, the variant is classified as pathogenic. GT-specific criteria applied: PVS1_Strong, PS3, PM2_supporiting, PM3, PP1, PP4_strong. |