Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000156694 | SCV000206415 | uncertain significance | not specified | 2014-07-17 | criteria provided, single submitter | clinical testing | The 33173-7A>G variant in TTN has not been previously reported in individuals wi th cardiomyopathy or in large population studies. This variant is located in the 3' splice region. Computational tools do not suggest an impact to splicing. How ever, this information is not predictive enough to rule pathogenicity. In summar y, the clinical significance of the 33173-7A>G variant is uncertain. |
Labcorp Genetics |
RCV000938441 | SCV001084251 | likely benign | Dilated cardiomyopathy 1G; Autosomal recessive limb-girdle muscular dystrophy type 2J | 2024-01-08 | criteria provided, single submitter | clinical testing | |
Gene |
RCV001657892 | SCV001881639 | benign | not provided | 2016-05-26 | criteria provided, single submitter | clinical testing | |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV000156694 | SCV003801233 | uncertain significance | not specified | 2023-01-21 | criteria provided, single submitter | clinical testing | Variant summary: TTN c.33173-7A>G alters a non-conserved nucleotide located close to a canonical splice site and therefore could affect mRNA splicing, leading to a significantly altered protein sequence. 4/4 computational tools predict no significant impact on normal splicing. However, these predictions have yet to be confirmed by functional studies. The variant allele was found at a frequency of 1.4e-05 in 139414 control chromosomes (gnomAD). The available data on variant occurrences in the general population are insufficient to allow any conclusion about variant significance. To our knowledge, no occurrence of c.33173-7A>G in individuals affected with Limb-Girdle Muscular Dystrophy, Type 2J and no experimental evidence demonstrating its impact on protein function have been reported. Two ClinVar submitters have assessed the variant since 2014: one classified the variant as likely benign and one as benign. Based on the evidence outlined above, the variant was classified as uncertain significance. |