Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV004821458 | SCV005442704 | uncertain significance | Snijders Blok-Campeau syndrome | 2023-06-22 | criteria provided, single submitter | clinical testing | The observed missense c.682G>A p.Val228Ile variant in CHD3 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Val228Ile variant is absent in gnomAD Exomes. This variant has not been submitted to the ClinVar database. Multiple lines of computational evidence Polyphen - Benign, SIFT - Tolerated and MutationTaster - Disease causing predicts conflicting evidence on protein structure and function for this variant. The reference amino acid on CHD3 gene is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. The amino acid Val at position 228 is changed to a Ile changing protein sequence and it might alter its composition and physico-chemical properties. For these reasons, this variant has been classified as Variant of Uncertain Significance VUS. |