Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Blueprint Genetics | RCV001074625 | SCV001240217 | likely pathogenic | Retinal dystrophy | 2019-01-29 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001388979 | SCV001590174 | pathogenic | PRPH2-related disorder | 2021-11-27 | 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: 143069). This missense change has been observed in individuals with autosomal dominant retinitis pigmentosa (PMID: 18310263, 30217183; Invitae). This variant is not present in population databases (gnomAD no frequency). This sequence change replaces glycine, which is neutral and non-polar, with aspartic acid, which is acidic and polar, at codon 137 of the PRPH2 protein (p.Gly137Asp). |
Department of Ophthalmology and Visual Sciences Kyoto University | RCV000132578 | SCV000172521 | pathogenic | Retinitis pigmentosa | no assertion criteria provided | not provided | Converted during submission to Pathogenic. | |
Leiden Open Variation Database | RCV001530286 | SCV001745044 | uncertain significance | not provided | 2021-04-06 | no assertion criteria provided | curation | Curator: Global Variome, with Curator vacancy. Submitter to LOVD: Manon Peeters. |