Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000019623 | SCV004293830 | pathogenic | Hereditary antithrombin deficiency | 2023-12-27 | criteria provided, single submitter | clinical testing | This sequence change replaces alanine, which is neutral and non-polar, with proline, which is neutral and non-polar, at codon 416 of the SERPINC1 protein (p.Ala416Pro). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individuals with antithrombin III deficiency (PMID: 2093312, 2776881, 24583439, 27838551, 33725558). It has also been observed to segregate with disease in related individuals. This variant is also known as p.Ala384Pro. ClinVar contains an entry for this variant (Variation ID: 18007). Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) has been performed at Invitae for this missense variant, however the output from this modeling did not meet the statistical confidence thresholds required to predict the impact of this variant on SERPINC1 protein function. For these reasons, this variant has been classified as Pathogenic. |
Ce |
RCV003886364 | SCV004704294 | likely pathogenic | not provided | 2023-12-01 | criteria provided, single submitter | clinical testing | SERPINC1: PM1, PM2, PP1:Moderate, PS4:Moderate, PP4 |
OMIM | RCV000019623 | SCV000039921 | pathogenic | Hereditary antithrombin deficiency | 1990-08-15 | no assertion criteria provided | literature only |