Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
3billion, |
RCV001775443 | SCV002012351 | likely pathogenic | Loeys-Dietz syndrome 1 | 2021-10-02 | criteria provided, single submitter | clinical testing | It is not observed in the gnomAD v2.1.1 dataset (PM2). A different missense change at the same codon (p.Gly218Ser) has been reported as pathogenic/likely pathogenic with strong evidence (ClinVar ID: VCV000488621.1, PM5). The variant was observed as assumed (i.e. paternity and maternity not confirmed) de novoo (3billion dataset, PM6). In silico tool predictions suggest damaging effect of the variant on gene or gene product (REVEL: 0.952, 3Cnet: 0.805, PP3). Therefore, this variant is classified as likely pathogenic according to the recommendation of ACMG/AMP guideline |