Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV003337857 | SCV004048224 | uncertain significance | Developmental and epileptic encephalopathy, 64 | criteria provided, single submitter | clinical testing | The missense variant c.2206G>A (p.Ala736Thr) in RHOBTB2 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Ala736Thr variant is novel (not in any individuals) in gnomAD Exomes and 1000 Genomes. The amino acid Ala at position 736 is changed to a Thr changing protein sequence and it might alter its composition and physico-chemical properties. In silico tools predict the variant to be tolerated. The residue is conserved across species. The amino acid change p.Ala736Thr in RHOBTB2 is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. For these reasons, this variant has been classified as Variant of Uncertain Significance (VUS). |