Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Biesecker Lab/Clinical Genomics Section, |
RCV000171817 | SCV000050829 | benign | not specified | 2013-06-24 | criteria provided, single submitter | research | |
Color Diagnostics, |
RCV001180808 | SCV001345829 | uncertain significance | Cardiomyopathy | 2023-04-28 | criteria provided, single submitter | clinical testing | This missense variant replaces glycine with tryptophan at codon 1885 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 not been identified 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. |
Ambry Genetics | RCV002345583 | SCV002651850 | uncertain significance | Cardiovascular phenotype | 2021-08-20 | criteria provided, single submitter | clinical testing | The p.G1885W variant (also known as c.5653G>T), located in coding exon 37 of the RYR2 gene, results from a G to T substitution at nucleotide position 5653. The glycine at codon 1885 is replaced by tryptophan, an amino acid with highly dissimilar properties. This alteration has been reported as a secondary cardiac variant in an exome cohort; however, clinical details are limited (Ng D et al. Circ Cardiovasc Genet, 2013 Aug;6:337-46). This amino acid position is not 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. |
All of Us Research Program, |
RCV003995654 | SCV004820155 | uncertain significance | Catecholaminergic polymorphic ventricular tachycardia | 2023-08-15 | criteria provided, single submitter | clinical testing | This missense variant replaces glycine with tryptophan at codon 1885 of the RYR2 protein. Computational prediction tools and conservation analyses are inconclusive regarding the impact of this variant on the protein function. Computational splicing tools suggest that this variant may not impact RNA splicing. To our knowledge, functional assays have not been performed for this variant nor has this variant been reported in individuals affected with cardiovascular disorders in the literature. This variant has not been identified in the general population by the Genome Aggregation Database (gnomAD). 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. |