Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001372953 | SCV001569649 | uncertain significance | not provided | 2021-10-15 | criteria provided, single submitter | clinical testing | This sequence change replaces isoleucine with serine at codon 360 of the DNASE2 protein (p.Ile360Ser). The isoleucine residue is weakly conserved and there is a large physicochemical difference between isoleucine and serine. This variant is present in population databases (rs554384572, ExAC 0.05%). This variant has not been reported in the literature in individuals affected with DNASE2-related conditions. 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 serine 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. |
Prevention |
RCV003416293 | SCV004117023 | uncertain significance | DNASE2-related disorder | 2022-10-27 | criteria provided, single submitter | clinical testing | The DNASE2 c.1079T>G variant is predicted to result in the amino acid substitution p.Ile360Ser. To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.039% of alleles in individuals of South Asian descent in gnomAD (http://gnomad.broadinstitute.org/variant/19-12986808-A-C). At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |
Ambry Genetics | RCV004037548 | SCV004860176 | likely benign | not specified | 2023-12-19 | criteria provided, single submitter | clinical testing | This alteration is classified as likely benign based on a combination of the following: seen in unaffected individuals, population frequency, intact protein function, lack of segregation with disease, co-occurrence, RNA analysis, in silico models, amino acid conservation, lack of disease association in case-control studies, and/or the mechanism of disease or impacted region is inconsistent with a known cause of pathogenicity. |