Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV004820745 | SCV005441449 | uncertain significance | not provided | 2024-06-27 | criteria provided, single submitter | clinical testing | Observed in an individual with thoracic aortic aneurysm who also harbored a variant in FBN1 (PMID: 34150014); Not observed at significant frequency in large population cohorts (gnomAD); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; Although located in a calcium-binding EGF-like domain of the FBN2 gene, it does not substitute or introduce a cysteine residue (PMID: 19006240, 18767143); This variant is associated with the following publications: (PMID: 19006240, 18767143, 34150014) |