Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001225254 | SCV001397511 | uncertain significance | Glanzmann thrombasthenia | 2023-06-01 | reviewed by expert panel | curation | This intronic variant, NM_000419.5(ITGA2B):c.2728-19T>C (p.=) occurs at an extremely low frequency (<1/10,000), with an overall allele frequency in gnomAD of 0.00001377 and an MAF of 0.00003751 (2/53,326 alleles) in the non-Finnish European population (PM2_supporting). To our knowledge, it has not been reported in the literature. The variant is predicted by SpliceAI to have no significant splicing motif alteration detected (BP4) and the nucleotide is not highly conserved (PhyloP score 0.099; BP7). In summary, this variant meets the criteria to be classified as variant of uncertain significance for autosomal recessive Glanzmann Thrombasthenia based on the ACMG/AMP criteria applied, as specified by the ClinGen PD VCEP: PM2_supporting, BP4, and BP7 (PD VCEP specifications version 2.1). |