Total submissions: 8
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002561131 | SCV001374889 | likely benign | Catecholaminergic polymorphic ventricular tachycardia 1 | 2024-12-24 | criteria provided, single submitter | clinical testing | |
Molecular Diagnostic Laboratory for Inherited Cardiovascular Disease, |
RCV001256776 | SCV001433220 | uncertain significance | not provided | 2019-12-09 | criteria provided, single submitter | clinical testing | |
Gene |
RCV001256776 | SCV002008806 | uncertain significance | not provided | 2023-08-22 | criteria provided, single submitter | clinical testing | Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis supports that this missense variant does not alter protein structure/function; Not located in one of the three hot-spot regions of the RYR2 gene, where the majority of pathogenic missense variants occur (Medeiros-Domingo et al., 2009); Has not been previously published as pathogenic or benign to our knowledge; This variant is associated with the following publications: (PMID: 19926015) |
Ambry Genetics | RCV002429871 | SCV002742496 | uncertain significance | Cardiovascular phenotype | 2023-01-24 | criteria provided, single submitter | clinical testing | The p.D849N variant (also known as c.2545G>A), located in coding exon 22 of the RYR2 gene, results from a G to A substitution at nucleotide position 2545. The aspartic acid at codon 849 is replaced by asparagine, an amino acid with highly similar properties. This variant was reported as an incidental finding in an exome cohort; however, clinical details were limited (Landstrom AP et al. Circ Arrhythm Electrophysiol, 2017 Apr;10:[Epub ahead of print]). This amino acid position is well conserved in available vertebrate species. In addition, this alteration is predicted to be tolerated by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Fulgent Genetics, |
RCV002484100 | SCV002790455 | uncertain significance | Arrhythmogenic right ventricular dysplasia 2; Catecholaminergic polymorphic ventricular tachycardia 1; Ventricular arrhythmias due to cardiac ryanodine receptor calcium release deficiency syndrome | 2021-09-30 | criteria provided, single submitter | clinical testing | |
Color Diagnostics, |
RCV003532891 | SCV004360539 | uncertain significance | Cardiomyopathy | 2023-11-28 | criteria provided, single submitter | clinical testing | This missense variant replaces aspartic acid with asparagine at codon 849 of the RYR2 protein. Computational prediction suggests that this variant may not impact protein structure and function (internally defined REVEL score threshold <= 0.5, PMID: 27666373). To our knowledge, functional studies have not been reported for this variant. This variant has not been reported in individuals affected with RYR2-related disorders in the literature. This variant has been identified in 4/280680 chromosomes in the general population by the Genome Aggregation Database (gnomAD). The available evidence is insufficient to determine the role of this variant in disease conclusively. Therefore, this variant is classified as a Variant of Uncertain Significance. |
All of Us Research Program, |
RCV004010632 | SCV004814865 | uncertain significance | Catecholaminergic polymorphic ventricular tachycardia | 2024-05-09 | criteria provided, single submitter | clinical testing | This missense variant replaces aspartic acid with asparagine at codon 849 of the RYR2 protein. Computational prediction suggests that this variant may not impact protein structure and function (internally defined REVEL score threshold <= 0.5, PMID: 27666373). To our knowledge, functional studies have not been reported for this variant. This variant has not been reported in individuals affected with cardiovascular disorders in the literature. This variant has been identified in 4/280680 chromosomes in the general population by the Genome Aggregation Database (gnomAD). The available evidence is insufficient to determine the role of this variant in disease conclusively. Therefore, this variant is classified as a Variant of Uncertain Significance. |
Juno Genomics, |
RCV004819240 | SCV005440618 | uncertain significance | Catecholaminergic polymorphic ventricular tachycardia 1; Ventricular arrhythmias due to cardiac ryanodine receptor calcium release deficiency syndrome | criteria provided, single submitter | clinical testing | PM2_Supporting |