Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001225249 | SCV001397501 | pathogenic | Glanzmann thrombasthenia | 2020-09-06 | reviewed by expert panel | curation | The splicing variant, c.1545-1del, has been observed in at least three compound heterozygous patients (PMIDs: 1060770 and 20819594, 32237906). The canonical splice variant is predicted to cause the skipping of exon 16, causing a frameshift and a premature stop codon in exon 18 of 30 leading to NMD.The variant is absent form ExAC, gnomAD, and 1000 Genomes. In summary, this variant meets criteria to be classified as Pathogenic for GT. GT-specific criteria applied: PVS1, PM2_Supporting, PP4_Moderate. |