Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000156911 | SCV000206632 | likely pathogenic | Primary dilated cardiomyopathy | 2014-11-25 | criteria provided, single submitter | clinical testing | The p.Ser20164X variant in TTN has not been previously reported in individuals w ith cardiomyopathy or in large population studies. This nonsense variant leads t o a premature termination codon at position 20164, which is predicted to lead to a truncated or absent protein. Nonsense and other truncating variants in TTN ar e strongly associated with DCM and the majority occur in the A-band (Herman 2012 , Pugh 2014), where this variant is located. In summary, although additional stu dies are required to fully establish its clinical significance, the p.Ser20164X variant is likely pathogenic. |