Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002619011 | SCV003496805 | uncertain significance | Senior-Loken syndrome 7; Bardet-Biedl syndrome 16 | 2022-04-28 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with leucine, which is neutral and non-polar, at codon 82 of the SDCCAG8 protein (p.Arg82Leu). This variant is present in population databases (rs577345357, gnomAD 0.003%). This variant has not been reported in the literature in individuals affected with SDCCAG8-related conditions. Algorithms developed to predict the effect of missense changes on protein structure and function (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be tolerated. 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. |
Prevention |
RCV004736276 | SCV005357188 | uncertain significance | SDCCAG8-related disorder | 2024-03-15 | no assertion criteria provided | clinical testing | The SDCCAG8 c.245G>T variant is predicted to result in the amino acid substitution p.Arg82Leu. This variant was reported in an individual with Bardet-Biedl syndrome; however, it was reported as a potential modifier allele, not a primary causative variant (Perea-Romero et al. 2022. PubMed ID: 35835773). This variant is reported in 0.0029% of alleles in individuals of Latino descent in gnomAD. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |