Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000652521 | SCV000774391 | uncertain significance | Tenorio syndrome | 2019-04-25 | criteria provided, single submitter | clinical testing | 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 missense changes on protein structure and function do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Benign"; Align-GVGD: "Class C0"). This variant has not been reported in the literature in individuals with RNF125-related disease. This variant is present in population databases (rs766520562, ExAC 0.02%). This sequence change replaces serine with cysteine at codon 16 of the RNF125 protein (p.Ser16Cys). The serine residue is highly conserved and there is a moderate physicochemical difference between serine and cysteine. |