Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Center For Human Genetics And Laboratory Diagnostics, |
RCV000515731 | SCV000611778 | likely pathogenic | Dilated cardiomyopathy 1G | 2017-08-09 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002527445 | SCV002963107 | pathogenic | Dilated cardiomyopathy 1G; Autosomal recessive limb-girdle muscular dystrophy type 2J | 2023-08-10 | criteria provided, single submitter | clinical testing | This premature translational stop signal has been observed in individuals with dilated cardiomyopathy (PMID: 31737537, 32659924; Invitae). For these reasons, this variant has been classified as Pathogenic. This variant is located in the A band of TTN (PMID: 25589632). Truncating variants in this region are significantly overrepresented in patients affected with dilated cardiomyopathy (PMID: 25589632). Truncating variants in this region have also been reported in individuals affected with autosomal recessive centronuclear myopathy (PMID: 23975875). ClinVar contains an entry for this variant (Variation ID: 446426). This variant is not present in population databases (gnomAD no frequency). This sequence change creates a premature translational stop signal (p.Tyr25182*) in the TTN gene. While this is not anticipated to result in nonsense mediated decay, it is expected to create a truncated TTN protein. |
Institute of Human Genetics, |
RCV000850282 | SCV000992457 | pathogenic | Primary dilated cardiomyopathy | no assertion criteria provided | clinical testing |