Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002971950 | SCV003286751 | uncertain significance | not provided | 2023-11-27 | criteria provided, single submitter | clinical testing | This sequence change replaces threonine, which is neutral and polar, with asparagine, which is neutral and polar, at codon 605 of the SETBP1 protein (p.Thr605Asn). This variant is present in population databases (no rsID available, gnomAD 0.003%). This variant has not been reported in the literature in individuals affected with SETBP1-related conditions. ClinVar contains an entry for this variant (Variation ID: 2073524). 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) has been performed at Invitae for this missense variant, however the output from this modeling did not meet the statistical confidence thresholds required to predict the impact of this variant on SETBP1 protein function. 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. |
Molecular Genetics Lab, |
RCV003883205 | SCV004697785 | likely benign | Schinzel-Giedion syndrome; Intellectual disability, autosomal dominant 29 | criteria provided, single submitter | clinical testing | ||
Ambry Genetics | RCV004958896 | SCV005495563 | uncertain significance | Inborn genetic diseases | 2024-11-25 | criteria provided, single submitter | clinical testing | The c.1814C>A (p.T605N) alteration is located in exon 4 (coding exon 3) of the SETBP1 gene. This alteration results from a C to A substitution at nucleotide position 1814, causing the threonine (T) at amino acid position 605 to be replaced by an asparagine (N). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |