Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000797705 | SCV000937280 | uncertain significance | Carney complex, type 1 | 2024-10-13 | criteria provided, single submitter | clinical testing | This sequence change replaces serine, which is neutral and polar, with arginine, which is basic and polar, at codon 5 of the PRKAR1A protein (p.Ser5Arg). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with PRKAR1A-related conditions. ClinVar contains an entry for this variant (Variation ID: 643895). Invitae Evidence Modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) indicates that this missense variant is not expected to disrupt PRKAR1A protein function with a negative predictive value of 95%. 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. |
Baylor Genetics | RCV004569551 | SCV005052479 | uncertain significance | Acrodysostosis 1 with or without hormone resistance | 2024-02-07 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV004649324 | SCV005151254 | uncertain significance | Hereditary cancer-predisposing syndrome | 2024-05-31 | criteria provided, single submitter | clinical testing | The p.S5R variant (also known as c.13A>C), located in coding exon 1 of the PRKAR1A gene, results from an A to C substitution at nucleotide position 13. The serine at codon 5 is replaced by arginine, an amino acid with dissimilar properties. This amino acid position is conserved. In addition, the in silico prediction for this alteration is inconclusive. Based on the available evidence, the clinical significance of this variant remains unclear. |