Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Invitae | RCV000007886 | SCV002117611 | pathogenic | Peutz-Jeghers syndrome | 2022-08-03 | criteria provided, single submitter | clinical testing | This premature translational stop signal has been observed in individual(s) with Peutz-Jeghers syndrome (PMID: 15200509, 32462036). This sequence change creates a premature translational stop signal (p.Tyr246*) in the STK11 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in STK11 are known to be pathogenic (PMID: 15188174, 16287113). This variant is not present in population databases (gnomAD no frequency). ClinVar contains an entry for this variant (Variation ID: 7460). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site. For these reasons, this variant has been classified as Pathogenic. |
OMIM | RCV000007886 | SCV000028091 | pathogenic | Peutz-Jeghers syndrome | 2004-07-01 | no assertion criteria provided | literature only |