Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000222488 | SCV000271280 | likely pathogenic | Primary dilated cardiomyopathy | 2015-03-20 | criteria provided, single submitter | clinical testing | The c.56693-1G>C variant in TTN has not been reported in individuals with cardio myopathy and was absent from large population studies. This variant occurs in th e invariant region (+/- 1,2) of the splice consensus sequence and is predicted t o cause altered splicing leading to an abnormal or absent protein. Splice and ot her truncating variants in TTN are strongly associated with DCM and the majority occur in the A-band (Herman 2012, LMM unpublished data), where this variant is located. In summary, although additional studies are required to fully establish its clinical significance, the c.56693-1G>C variant is likely pathogenic. |