Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000538044 | SCV000630820 | uncertain significance | Walker-Warburg congenital muscular dystrophy | 2021-10-03 | criteria provided, single submitter | clinical testing | This sequence change replaces phenylalanine with valine at codon 20 of the FKTN protein (p.Phe20Val). The phenylalanine residue is highly conserved and there is a small physicochemical difference between phenylalanine and valine. This variant is not present in population databases (ExAC no frequency). This missense change has been observed in individual(s) with LBBB, PVC, atrial fibrillation, nonischemic cardiomyopathy (Invitae). 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 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. |
Eurofins Ntd Llc |
RCV000730719 | SCV000858480 | uncertain significance | not provided | 2017-12-05 | criteria provided, single submitter | clinical testing | |
Gene |
RCV000730719 | SCV002003652 | uncertain significance | not provided | 2021-04-06 | criteria provided, single submitter | clinical testing | Not observed in large population cohorts (Lek et al., 2016); 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 |
Revvity Omics, |
RCV000730719 | SCV003832678 | uncertain significance | not provided | 2019-10-01 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV004619323 | SCV005118148 | uncertain significance | Cardiovascular phenotype | 2024-03-16 | criteria provided, single submitter | clinical testing | The p.F20V variant (also known as c.58T>G), located in coding exon 1 of the FKTN gene, results from a T to G substitution at nucleotide position 58. The phenylalanine at codon 20 is replaced by valine, an amino acid with highly similar properties. This amino acid position is conserved. In addition, this alteration is predicted to be deleterious by in silico analysis. Based on the available evidence, the clinical significance of this alteration remains unclear. |