Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Prevention |
RCV000721777 | SCV000852914 | likely pathogenic | not provided | 2017-03-10 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001868914 | SCV002229328 | pathogenic | Cerebral cavernous malformation 2 | 2020-12-08 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Disruption of this splice site has been observed in individual(s) with cerebral cavernous malformations (PMID: 17277691, Invitae). This variant is also known as IVS 3 G-1T. ClinVar contains an entry for this variant (Variation ID: 590652). This variant is not present in population databases (ExAC no frequency). This sequence change affects an acceptor splice site in intron 3 of the CCM2 gene. It is expected to disrupt RNA splicing. Variants that disrupt the donor or acceptor splice site typically lead to a loss of protein function (PMID: 16199547), and loss-of-function variants in CCM2 are known to be pathogenic (PMID: 18300272, 24689081). |