Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Invitae | RCV001386418 | SCV001586613 | pathogenic | Peutz-Jeghers syndrome | 2020-10-09 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Loss-of-function variants in STK11 are known to be pathogenic (PMID: 15188174, 16287113). A different variant (c.354C>G) giving rise to the same protein effect observed here (p.Tyr118*) has been determined to be pathogenic (PMID: 15863673, 30092773). This suggests that this variant is also likely to be causative of disease. This variant is not present in population databases (ExAC no frequency). This sequence change creates a premature translational stop signal (p.Tyr118*) in the STK11 gene. It is expected to result in an absent or disrupted protein product. |