Total submissions: 8
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000036796 | SCV000060451 | uncertain significance | not specified | 2011-09-27 | criteria provided, single submitter | clinical testing | The Ile2770Thr variant (RYR2) has not been reported in the literature and has no t been previously detected by our laboratory. Isoleucine (Ile) at position 2770 is not completely conserved in evolutionarily distant species (zebrafish carries a difference amino acid), raising the possibility that this change may be toler ated. Computational predications are mixed, though the accuracy of these tools i s unknown. In summary, additional data is needed to clarify the significance of this variant. |
Illumina Laboratory Services, |
RCV001095886 | SCV001252063 | uncertain significance | Arrhythmogenic right ventricular dysplasia 2 | 2017-04-28 | criteria provided, single submitter | clinical testing | This variant was observed as part of a predisposition screen in an ostensibly healthy population. A literature search was performed for the gene, cDNA change, and amino acid change (where applicable). No publications were found based on this search. Allele frequency data from public databases did not allow this variant to be ruled in or out of causing disease. Therefore, this variant is classified as a variant of unknown significance. |
Illumina Laboratory Services, |
RCV001095887 | SCV001252064 | uncertain significance | Catecholaminergic polymorphic ventricular tachycardia 1 | 2017-04-28 | criteria provided, single submitter | clinical testing | This variant was observed as part of a predisposition screen in an ostensibly healthy population. A literature search was performed for the gene, cDNA change, and amino acid change (where applicable). No publications were found based on this search. Allele frequency data from public databases did not allow this variant to be ruled in or out of causing disease. Therefore, this variant is classified as a variant of unknown significance. |
Color Diagnostics, |
RCV001175860 | SCV001339639 | uncertain significance | Cardiomyopathy | 2023-04-07 | criteria provided, single submitter | clinical testing | This variant is located in the RYR2 protein. Computational prediction is inconclusive regarding the impact of this variant on protein structure and function (internally defined REVEL score threshold 0.5 < inconclusive < 0.7, 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 5/185468 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. |
Fulgent Genetics, |
RCV002496576 | SCV002814096 | uncertain significance | Arrhythmogenic right ventricular dysplasia 2; Catecholaminergic polymorphic ventricular tachycardia 1; Ventricular arrhythmias due to cardiac ryanodine receptor calcium release deficiency syndrome | 2021-11-16 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001095887 | SCV003439854 | likely benign | Catecholaminergic polymorphic ventricular tachycardia 1 | 2025-01-14 | criteria provided, single submitter | clinical testing | |
All of Us Research Program, |
RCV003996303 | SCV004823276 | uncertain significance | Catecholaminergic polymorphic ventricular tachycardia | 2024-01-11 | criteria provided, single submitter | clinical testing | This variant is located in the RYR2 protein. Computational prediction is inconclusive regarding the impact of this variant on protein structure and function (internally defined REVEL score threshold 0.5 < inconclusive < 0.7, 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 5/185468 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. |
Ambry Genetics | RCV004017312 | SCV004849270 | uncertain significance | Cardiovascular phenotype | 2019-10-08 | criteria provided, single submitter | clinical testing | The c.8309T>C (p.I2770T) alteration is located in exon 56 (coding exon 56) of the RYR2 gene. This alteration results from a T to C substitution at nucleotide position 8309, causing the isoleucine (I) at amino acid position 2770 to be replaced by a threonine (T). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |