Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Counsyl | RCV000668336 | SCV000792916 | pathogenic | Primary hyperoxaluria, type I | 2017-07-24 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002530741 | SCV003520198 | pathogenic | not provided | 2023-09-04 | criteria provided, single submitter | clinical testing | A different variant (c.466G>A) giving rise to the same protein effect has been determined to be pathogenic (PMID: 17460142, 23810941, 27935012). This suggests that this variant is also likely to be causative of disease. This variant is not present in population databases (gnomAD no frequency). This sequence change replaces glycine, which is neutral and non-polar, with arginine, which is basic and polar, at codon 156 of the AGXT protein (p.Gly156Arg). For these reasons, this variant has been classified as Pathogenic. 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) performed at Invitae indicates that this missense variant is expected to disrupt AGXT protein function. ClinVar contains an entry for this variant (Variation ID: 552979). |