Total submissions: 7
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000559374 | SCV000643402 | uncertain significance | Dilated cardiomyopathy 1G; Autosomal recessive limb-girdle muscular dystrophy type 2J | 2017-01-31 | criteria provided, single submitter | clinical testing | |
ARUP Laboratories, |
RCV000756856 | SCV000884810 | uncertain significance | not provided | 2018-06-04 | criteria provided, single submitter | clinical testing | The TTN: c.49180C>T; p.Arg16394Trp variant is rare in the general population (<1% allele frequency in the Genome Aggregation Database) and has not been reported in the medical literature in association with dilated cardiomyopathy (DCM) or other TTN-related disease. The clinical relevance of rare missense variants in this gene, which are identified on average once per individual sequenced in affected populations (Herman 2012), is not well understood. While the clinical significance of such variants is considered uncertain, evidence suggests that the vast majority of missense variants do not contribute to the clinical outcome of DCM (Begay 2015). Given the available evidence, the clinical significance of the p.Arg16394Trp variant cannot be determined with certainty. |
Gene |
RCV000756856 | SCV001783278 | likely benign | not provided | 2019-12-02 | criteria provided, single submitter | clinical testing | |
CHEO Genetics Diagnostic Laboratory, |
RCV001798885 | SCV002042548 | uncertain significance | Cardiomyopathy | 2020-08-13 | criteria provided, single submitter | clinical testing | |
Revvity Omics, |
RCV000756856 | SCV003819825 | uncertain significance | not provided | 2023-01-12 | criteria provided, single submitter | clinical testing | |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV005239179 | SCV005884271 | uncertain significance | not specified | 2024-12-02 | criteria provided, single submitter | clinical testing | Variant summary: TTN c.49180C>T (p.Arg16394Trp) results in a non-conservative amino acid change in the encoded protein sequence. Three of three in-silico tools predict a damaging effect of the variant on protein function. The variant allele was found at a frequency of 8.1e-06 in 248192 control chromosomes. The available data on variant occurrences in the general population are insufficient to allow any conclusion about variant significance. c.49180C>T has been reported in the heterozygous state in the literature in at least 1 individual affected with dilated cardiomyopathy (example, Lian_2023). These report(s) do not provide unequivocal conclusions about association of the variant with TTN-related conditions. To our knowledge, no experimental evidence demonstrating an impact on protein function has been reported. The following publication has been ascertained in the context of this evaluation (PMID: 37461109). ClinVar contains an entry for this variant (Variation ID: 467293). Based on the evidence outlined above, the variant was classified as uncertain significance. |
Molecular Diagnostic Laboratory for Inherited Cardiovascular Disease, |
RCV005239179 | SCV006066398 | likely benign | not specified | 2025-04-09 | criteria provided, single submitter | clinical testing |