Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV000851834 | SCV001478486 | uncertain significance | Glanzmann thrombasthenia | 2023-12-19 | reviewed by expert panel | curation | The intronic variant c.624+5G>A has been reported in one compound heterozygous GT patient (PMID: 31064749) with the second variant of Asp396Asn (classified Pathogenic by PD-EP; PM3_supporting). It occurs at an extremely low frequency, (below the PM2_supporting <1/10,000 threshold) with a MAF of 0.00003729 (44/1,180,018) in the gnomADv4.0 non-Finnish European population. SpliceAI predicts a loss of the donor splice site (delta score 0.77; PP3). In summary, this variant meets criteria to be classified as uncertain significance. GT-specific criteria applied: PM2_supporting, PM3_supporting, PP3. |
NIHR Bioresource Rare Diseases, |
RCV000851834 | SCV000899834 | uncertain significance | Glanzmann thrombasthenia | 2019-02-01 | criteria provided, single submitter | research |