Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV004785811 | SCV005400865 | uncertain significance | Coffin-Siris syndrome 8 | 2023-06-22 | criteria provided, single submitter | clinical testing | The observed missense c.2953A>G(p.Met985Val) variant in SMARCC2 gene has not been reported previously as a pathogenic variant nor a benign variant, to our knowledge. The p.Met985Val variant is absent in gnomAD Exomes. This variant has not been submitted to the ClinVar database. Multiple lines of computational evidences (Polyphen - Benign, SIFT - Tolerated and MutationTaster - Disease causing) predict conflicting evidence on protein structure and function for this variant. The reference amino acid change at this position on SMARCC2 gene is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. The amino acid Met at position 985 is changed to a Val changing protein sequence and it might alter its composition and physico-chemical properties. For these reasons, this variant has been classified as a Variant of Uncertain Significance (VUS). |