Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000399152 | SCV000329217 | pathogenic | not provided | 2015-11-18 | criteria provided, single submitter | clinical testing | The Q72X nonsense variant in the CCM2 gene is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. The Q72X variant was not observed in approximately 6,500 individuals of European and African American ancestry in the NHLBI Exome Sequencing Project, indicating it is not a common benign variant in these populations. Given the available evidence, we interpret Q72X as a pathogenic variant. |
Labcorp Genetics |
RCV001855050 | SCV002229882 | pathogenic | Cerebral cavernous malformation 2 | 2021-05-26 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. This variant has not been reported in the literature in individuals with CCM2-related conditions. ClinVar contains an entry for this variant (Variation ID: 279739). This variant is not present in population databases (ExAC no frequency). This sequence change creates a premature translational stop signal (p.Gln72*) in the CCM2 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in CCM2 are known to be pathogenic (PMID: 18300272, 24689081). |