Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV005208398 | SCV005849431 | uncertain significance | Emery-Dreifuss muscular dystrophy 5, autosomal dominant | 2023-06-22 | criteria provided, single submitter | clinical testing | The missense variant [c.6011G>C; p.Gly2004Ala] in the SYNE2 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Gly2004Ala variant is absent in gnomAD Exomes. This variant has not been submitted in the ClinVar database. Multiple lines of computational evidences (Polyphen - Probably damaging, SIFT - Damaging and MutationTaster - Disease causing) predict a damaging effect on protein structure and function for this variant. The reference amino acid change at this position on SYNE2 gene is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. The amino acid Gly at position 2004 is changed to a Ala changing protein sequence and it might alter its composition and physicochemical properties. For these reasons, this variant has been classified as Variant of Uncertain Significance (VUS). |