Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Invitae | RCV000806137 | SCV000946119 | pathogenic | Cerebral cavernous malformation 2 | 2018-09-10 | 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 CCM2 are known to be pathogenic (PMID: 14624391, 17160895). This variant has been observed in an individual affected with cerebral cavernous malformations (Invitae). This variant is not present in population databases (ExAC no frequency). This sequence change affects a donor splice site in intron 6 of the CCM2 gene. It is expected to disrupt RNA splicing and likely results in an absent or disrupted protein product. |