Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000812392 | SCV000952703 | uncertain significance | not provided | 2025-02-03 | criteria provided, single submitter | clinical testing | This sequence change replaces tryptophan, which is neutral and slightly polar, with cysteine, which is neutral and slightly polar, at codon 92 of the NTHL1 protein (p.Trp92Cys). This variant is present in population databases (rs372698989, gnomAD 0.008%). This missense change has been observed in individual(s) with adenomatous polyps (PMID: 37834005). ClinVar contains an entry for this variant (Variation ID: 656073). An algorithm developed to predict the effect of missense changes on protein structure and function (PolyPhen-2) suggests that this variant is likely to be disruptive. 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. |
Sema4, |
RCV002255533 | SCV002528948 | uncertain significance | Hereditary cancer-predisposing syndrome | 2021-08-25 | criteria provided, single submitter | curation | |
Ambry Genetics | RCV002255533 | SCV002747203 | uncertain significance | Hereditary cancer-predisposing syndrome | 2024-09-16 | criteria provided, single submitter | clinical testing | The p.W92C variant (also known as c.276G>C), located in coding exon 2 of the NTHL1 gene, results from a G to C substitution at nucleotide position 276. The tryptophan at codon 92 is replaced by cysteine, an amino acid with highly dissimilar properties. This amino acid position is highly conserved in available vertebrate species. In addition, this alteration is predicted to be deleterious by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Gene |
RCV000812392 | SCV004022828 | uncertain significance | not provided | 2023-07-18 | criteria provided, single submitter | clinical testing | Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; Has not been previously published as pathogenic or benign to our knowledge |
Quest Diagnostics Nichols Institute San Juan Capistrano | RCV000812392 | SCV004222195 | uncertain significance | not provided | 2022-12-28 | criteria provided, single submitter | clinical testing | The variant has not been reported in the published literature. The frequency of this variant in the general population, 0.000023 (3/128714 chromosomes, http://gnomad.broadinstitute.org), is uninformative in assessment of its pathogenicity. Analysis of this variant using bioinformatics tools for the prediction of the effect of amino acid changes on protein structure and function yielded predictions that this variant is damaging. Based on the available information, we are unable to determine the clinical significance of this variant. |
Baylor Genetics | RCV004569680 | SCV005053742 | uncertain significance | Familial adenomatous polyposis 3 | 2024-01-11 | criteria provided, single submitter | clinical testing |