Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Mendelics | RCV000989173 | SCV001139393 | uncertain significance | Leber congenital amaurosis 1 | 2019-05-28 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001858701 | SCV002306667 | uncertain significance | Cone-rod dystrophy 13; Leber congenital amaurosis 6 | 2022-05-03 | criteria provided, single submitter | clinical testing | This variant is present in population databases (no rsID available, gnomAD 0.0009%). This missense change has been observed in individuals with Leber congenital amaurosis (PMID: 32865313). ClinVar contains an entry for this variant (Variation ID: 803003). Algorithms developed to predict the effect of missense changes on protein structure and function are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Tolerated"; PolyPhen-2: "Probably Damaging"; Align-GVGD: "Class C0"). 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. This sequence change replaces glycine, which is neutral and non-polar, with glutamic acid, which is acidic and polar, at codon 671 of the RPGRIP1 protein (p.Gly671Glu). |