Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Invitae | RCV001227457 | SCV001399818 | uncertain significance | Senior-Loken syndrome 7; Bardet-Biedl syndrome 16 | 2022-08-22 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with histidine, which is basic and polar, at codon 82 of the SDCCAG8 protein (p.Arg82His). This variant is present in population databases (rs577345357, gnomAD 0.006%). This variant has not been reported in the literature in individuals affected with SDCCAG8-related conditions. ClinVar contains an entry for this variant (Variation ID: 954914). Algorithms developed to predict the effect of missense changes on protein structure and function output the following: SIFT: "Tolerated"; PolyPhen-2: "Benign"; Align-GVGD: "Class C0". The histidine amino acid residue is found in multiple mammalian species, which suggests that this missense change does not adversely affect protein function. 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. |
Genetic Services Laboratory, |
RCV001819925 | SCV002065717 | uncertain significance | not specified | 2018-09-27 | criteria provided, single submitter | clinical testing | |
Prevention |
RCV004538476 | SCV004709012 | uncertain significance | SDCCAG8-related disorder | 2024-03-01 | criteria provided, single submitter | clinical testing | The SDCCAG8 c.245G>A variant is predicted to result in the amino acid substitution p.Arg82His. To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.0062% of alleles in individuals of European (Non-Finnish) descent in gnomAD. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |