Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000609565 | SCV000712638 | uncertain significance | not specified | 2016-11-23 | criteria provided, single submitter | clinical testing | The p.Lys48Arg variant in STK11 has not been previously reported in individuals with Peutz-Jeghers syndrome or in large population studies. Computational predic tion tools and conservation analysis do not provide strong support for or agains t an impact to the protein. In summary, the clinical significance of the p.Lys48 Arg variant is uncertain. |
Color Diagnostics, |
RCV000772687 | SCV000905946 | uncertain significance | Hereditary cancer-predisposing syndrome | 2019-04-25 | criteria provided, single submitter | clinical testing | |
Invitae | RCV001321016 | SCV001511829 | uncertain significance | Peutz-Jeghers syndrome | 2022-07-12 | criteria provided, single submitter | clinical testing | This variant is not present in population databases (gnomAD no frequency). 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. Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site. Experimental studies have shown that this missense change does not substantially affect STK11 function (PMID: 18687677, 23577667, 30594553, 31217475, 31554794). 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. ClinVar contains an entry for this variant (Variation ID: 505434). This variant has not been reported in the literature in individuals affected with STK11-related conditions. This sequence change replaces lysine, which is basic and polar, with arginine, which is basic and polar, at codon 48 of the STK11 protein (p.Lys48Arg). |
Genome- |
RCV001321016 | SCV002057747 | uncertain significance | Peutz-Jeghers syndrome | 2021-07-15 | criteria provided, single submitter | clinical testing |