Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001290485 | SCV001478523 | pathogenic | Glanzmann thrombasthenia | 2020-11-10 | reviewed by expert panel | curation | The c.799+2T>C splice variant has been reported in at least one compound heterozygous proband (PMID: 29675921) with a phenotype highly specific to GT. It is predicted to cause the in-frame skipping of exon 7 which is part of the critical ligand binding domain. This variant is absent from controls in gnomAD, ExAC, and 1000 genomes. In summary, this variant meets criteria to be classified as Pathogenic for GT. GT-specific criteria applied: PVS1_Strong, PM2_Supporting, PM3_Supporting, and PP4_Strong. |