Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000546620 | SCV000630812 | uncertain significance | Walker-Warburg congenital muscular dystrophy | 2021-08-24 | criteria provided, single submitter | clinical testing | This sequence change replaces serine with arginine at codon 352 of the FKTN protein (p.Ser352Arg). The serine residue is highly conserved and there is a moderate physicochemical difference between serine and arginine. This variant is present in population databases (rs149085844, ExAC 0.005%). This variant has not been reported in the literature in individuals affected with FKTN-related conditions. 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. Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site. 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. |
Ambry Genetics | RCV004023803 | SCV005037351 | uncertain significance | Cardiovascular phenotype | 2024-02-06 | criteria provided, single submitter | clinical testing | The p.S352R variant (also known as c.1056C>G), located in coding exon 8 of the FKTN gene, results from a C to G substitution at nucleotide position 1056. The serine at codon 352 is replaced by arginine, an amino acid with dissimilar 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. |
Natera, |
RCV000546620 | SCV002081964 | uncertain significance | Walker-Warburg congenital muscular dystrophy | 2020-01-14 | no assertion criteria provided | clinical testing |