Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001882585 | SCV002235767 | pathogenic | PRPH2-related disorder | 2023-08-04 | criteria provided, single submitter | clinical testing | 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 PRPH2 protein function. ClinVar contains an entry for this variant (Variation ID: 1175266). This missense change has been observed in individuals with autosomal dominant retinitis pigmentosa (PMID: 9587927, 9673478, 10193525, 25447119). It has also been observed to segregate with disease in related individuals. This variant is not present in population databases (gnomAD no frequency). This sequence change replaces phenylalanine, which is neutral and non-polar, with leucine, which is neutral and non-polar, at codon 211 of the PRPH2 protein (p.Phe211Leu). |
Dept Of Ophthalmology, |
RCV003888299 | SCV004707341 | likely pathogenic | Retinal dystrophy | 2023-10-01 | criteria provided, single submitter | research | |
Institute of Human Genetics, |
RCV003888299 | SCV005070586 | likely pathogenic | Retinal dystrophy | 2016-01-01 | criteria provided, single submitter | clinical testing | |
Leiden Open Variation Database | RCV001530319 | SCV001745092 | uncertain significance | not provided | 2019-07-24 | no assertion criteria provided | curation | Curator: Global Variome, with Curator vacancy. Submitter to LOVD: Yoshito Koyanagi. |