Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
New York Genome Center | RCV002468534 | SCV002764544 | uncertain significance | Wolfram syndrome 1; Wolfram-like syndrome | 2020-11-02 | criteria provided, single submitter | clinical testing | The c.1620G>T, p.Trp540Cys missense variant identified in WFS1 has not been reported in the literature. This variant is not reported in gnomAD database, indicating this is a rare allele. This substitution occurs at a position that is conserved across species, and in silico analysis predicts this variant is probably damaging to the protein structure/function [PMID: 27268795]. Two allelic variants with different amino acid changes (c.1618T>G, p.Trp540Gly and c.1619G>A, p.Trp540Ter) have been reported in the patients with Wolfram syndrome [PMID: 31391115, 21564155]. Based on the available evidence, the variant c.1620G>T, p.Trp540Cys in the WFS1 gene is classified as a Variant of Uncertain Significance. |