Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Broad Center for Mendelian Genomics, |
RCV002789975 | SCV003761250 | uncertain significance | Familial isolated congenital asplenia | 2023-01-25 | criteria provided, single submitter | curation | The heterozygous p.Asn164Ile variant in RPSA was identified by our study in one individual with isolated congenital asplenia. The p.Asn164Ile variant in RPSA has not been previously reported in individuals with isolated congenital asplenia. This variant was absent from large population studies. Computational prediction tools and conservation analyses suggest that this variant may impact the protein, though this information is not predictive enough to determine pathogenicity. In summary, the clinical significance of the p.Asn164Ile variant is uncertain. ACMG/AMP Criteria applied: PM2_Supporting, PP3 (Richards 2015). |