Total submissions: 11
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Biesecker Lab/Clinical Genomics Section, |
RCV000172660 | SCV000054983 | likely benign | not provided | 2013-06-24 | criteria provided, single submitter | research | |
Laboratory for Molecular Medicine, |
RCV000152301 | SCV000201168 | uncertain significance | not specified | 2014-08-27 | criteria provided, single submitter | clinical testing | Variant classified as Uncertain Significance - Favor Benign. The Ser15890Arg var iant in TTN has not been previously reported in individuals with cardiomyopathy, but has been identified in 0.1% (10/8194) of European American chromosomes by t he NHLBI Exome Sequencing Project (http://evs.gs.washington.edu/EVS/; dbSNP rs20 0550947). Computational prediction tools and conservation analysis do not provid e strong support for or against an impact to the protein. In summary, while the clinical significance of the Ser15890Arg variant is uncertain, its frequency sug gests that it is more likely to be benign. |
Gene |
RCV000172660 | SCV000237316 | likely benign | not provided | 2020-10-05 | criteria provided, single submitter | clinical testing | This variant is associated with the following publications: (PMID: 23861362) |
Eurofins Ntd Llc |
RCV000172660 | SCV000333474 | uncertain significance | not provided | 2015-07-31 | criteria provided, single submitter | clinical testing | |
Invitae | RCV000476468 | SCV000542339 | uncertain significance | Dilated cardiomyopathy 1G; Autosomal recessive limb-girdle muscular dystrophy type 2J | 2017-12-29 | criteria provided, single submitter | clinical testing | |
Ce |
RCV000172660 | SCV001152871 | uncertain significance | not provided | 2023-11-01 | criteria provided, single submitter | clinical testing | |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV000152301 | SCV001821441 | likely benign | not specified | 2021-08-30 | criteria provided, single submitter | clinical testing | Variant summary: TTN c.47670C>G (p.Ser15890Arg) results in a non-conservative amino acid change located in the A-band domain of the encoded protein sequence. Three of five in-silico tools predict a damaging effect of the variant on protein function. The variant allele was found at a frequency of 0.00041 in 277010 control chromosomes, predominantly at a frequency of 0.0007 within the Non-Finnish European subpopulation in the gnomAD database. The observed variant frequency within Non-Finnish European control individuals in the gnomAD database is approximately 2 fold of the estimated maximal expected allele frequency for a pathogenic variant in TTN causing Dilated Cardiomyopathy phenotype (0.00039), strongly suggesting that the variant is a benign polymorphism found primarily in populations of Non-Finnish European origin. c.47670C>G has been reported in the literature in one individual affected with Dilated Cardiomyopathy (Roggenbuck_2019). The report does not provide unequivocal conclusions about association of the variant with Dilated Cardiomyopathy. Additionally, one co-occurrence with another pathogenic variant has been reported (exons 346-362 deletion; Roggenbuck_2019), providing supporting evidence for a benign role. To our knowledge, no experimental evidence demonstrating an impact on protein function has been reported. Four ClinVar submitters (evaluation after 2014) cite the variant as uncertain significance (n=3) and likely benign (n=1). Based on the evidence outlined above, the variant was classified as likely benign. |
CHEO Genetics Diagnostic Laboratory, |
RCV001798480 | SCV002042543 | likely benign | Cardiomyopathy | 2020-03-16 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV002433662 | SCV002747655 | likely benign | Cardiovascular phenotype | 2020-01-02 | criteria provided, single submitter | clinical testing | This alteration is classified as likely benign based on a combination of the following: seen in unaffected individuals, population frequency, intact protein function, lack of segregation with disease, co-occurrence, RNA analysis, in silico models, amino acid conservation, lack of disease association in case-control studies, and/or the mechanism of disease or impacted region is inconsistent with a known cause of pathogenicity. |
Revvity Omics, |
RCV000172660 | SCV003821069 | uncertain significance | not provided | 2023-02-22 | criteria provided, single submitter | clinical testing | |
Mayo Clinic Laboratories, |
RCV000172660 | SCV004225859 | uncertain significance | not provided | 2022-03-17 | criteria provided, single submitter | clinical testing |