Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001961799 | SCV002248100 | uncertain significance | Syndromic X-linked intellectual disability 14 | 2023-09-08 | criteria provided, single submitter | clinical testing | In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is not expected to disrupt UPF3B protein function. ClinVar contains an entry for this variant (Variation ID: 1469888). This variant has not been reported in the literature in individuals affected with UPF3B-related conditions. This variant is present in population databases (rs185347914, gnomAD 0.02%), including at least one homozygous and/or hemizygous individual. This sequence change replaces arginine, which is basic and polar, with glutamine, which is neutral and polar, at codon 350 of the UPF3B protein (p.Arg350Gln). |