Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV004018360 | SCV004848106 | likely pathogenic | Congenital long QT syndrome | 2019-01-28 | criteria provided, single submitter | clinical testing | The p.Val377fs variant in KCNH2 has not been previously reported in individuals with Long QT syndrome or in large population studies. This variant is predicted to cause a frameshift, which alters the protein’s amino acid sequence beginning at position 377 and leads to a premature termination codon 11 amino acids downstream. This alteration is then predicted to lead to a truncated or absent protein. In summary, although additional studies are required to fully establish its clinical significance, the p.Val377fs variant is likely pathogenic. |