Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV000492404 | SCV000580900 | pathogenic | Hereditary cancer-predisposing syndrome | 2013-01-09 | criteria provided, single submitter | clinical testing | Other acmg-defined mutation (i.e. initiation codon or gross deletion) |
Invitae | RCV000807365 | SCV000947413 | pathogenic | Peutz-Jeghers syndrome | 2018-08-15 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Donor and acceptor splice site variants typically lead to a loss of protein function (PMID: 16199547), and loss-of-function variants in STK11 are known to be pathogenic (PMID: 15188174, 16287113). Disruption of this splice site has been observed in individuals affected with Peutz Jeagher syndrome (PMID: 26607058). ClinVar contains an entry for this variant (Variation ID: 428756). This variant is not present in population databases (ExAC no frequency). This sequence change affects a donor splice site in intron 6 of the STK11 gene. It is expected to disrupt RNA splicing and likely results in an absent or disrupted protein product. |
Genome- |
RCV000807365 | SCV002057384 | pathogenic | Peutz-Jeghers syndrome | 2021-07-15 | criteria provided, single submitter | clinical testing |