Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001064940 | SCV001229877 | uncertain significance | PRPH2-related disorder | 2023-04-10 | criteria provided, single submitter | clinical testing | In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. 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: 858947). This missense change has been observed in individual(s) with clinical features of PRPH2-related conditions (PMID: 22863181, 32531846). This variant is present in population databases (rs146686238, gnomAD 0.1%). This sequence change replaces tryptophan, which is neutral and slightly polar, with cysteine, which is neutral and slightly polar, at codon 25 of the PRPH2 protein (p.Trp25Cys). |
NEI Ophthalmic Genomics Laboratory, |
RCV001250339 | SCV001424659 | uncertain significance | Retinitis pigmentosa | 2020-01-07 | criteria provided, single submitter | clinical testing | The variant NM_000322.4:c.75G>C in the PRPH2 gene has been previously studied(PMID 22863181). We found this variant in 1 patient(s) in a PRPH2 cohort study (Reeves et al. 2020). This variant is listed in dbSNP and/or HGMD (rs146686238,CM127070). It is present in gnomAD browser (AF 0.0000609). This variant is not already listed in ClinVar. It is not enriched in the PRPH2 disease cohort. We invoked ACMG criteria [PM2, PP3] and classified NM_000322.4:c.75G>C in the PRPH2 gene as a Variant of Uncertain Significance. |
Leiden Open Variation Database | RCV001530275 | SCV001745030 | uncertain significance | not provided | 2021-04-06 | no assertion criteria provided | curation | Curator: Global Variome, with Curator vacancy. Submitter to LOVD: Manon Peeters. |