Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001882806 | SCV002194880 | pathogenic | not provided | 2023-11-05 | criteria provided, single submitter | clinical testing | This sequence change replaces methionine, which is neutral and non-polar, with arginine, which is basic and polar, at codon 252 of the TYR protein (p.Met252Arg). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individual(s) with clinical features of oculocutaneous albinism (PMID: 29345414; Invitae). In at least one individual the data is consistent with being in trans (on the opposite chromosome) from a pathogenic variant. ClinVar contains an entry for this variant (Variation ID: 1301877). 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 TYR protein function with a positive predictive value of 80%. For these reasons, this variant has been classified as Pathogenic. |
Gene |
RCV001882806 | SCV003805552 | uncertain significance | not provided | 2022-08-18 | criteria provided, single submitter | clinical testing | In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; Not observed at significant frequency in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 29345414, 35027574) |
Ce |
RCV001882806 | SCV004010109 | pathogenic | not provided | 2023-08-01 | criteria provided, single submitter | clinical testing | TYR: PM3:Very Strong, PM2, PM5, PP4 |
Rare Disease Group, |
RCV002226431 | SCV001984755 | likely pathogenic | Tyrosinase-negative oculocutaneous albinism | no assertion criteria provided | research |