Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV004585154 | SCV005073858 | uncertain significance | Palmoplantar keratoderma-esophageal carcinoma syndrome | criteria provided, single submitter | clinical testing | The observed missense variant c.65G>A (p.Arg22Gln) has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Arg22Gln variant is presesnt with an allele frequency of 0.006% in the gnomAD exomes database. This variant has not been submitted to the ClinVar database. The amino acid change p.Arg22Gln in RHBDF2 is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. The amino acid Arg at position 22 is changed to a Gln 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). |