Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV002464069 | SCV002759210 | pathogenic | not provided | 2022-11-25 | criteria provided, single submitter | clinical testing | Occurs in the triple helical domain and replaces a glycine in a canonical Gly-X-Y repeat; missense substitution of a canonical glycine residue is expected to disrupt normal protein folding and function, and this is an established mechanism of disease (Jovanovic 2021); 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; Also known as p.(G691C); This variant is associated with the following publications: (PMID: 35583673, 34617103, 1953667, 34007986) |
OMIM | RCV000018833 | SCV000039116 | pathogenic | Osteogenesis imperfecta, perinatal lethal | 1988-08-25 | no assertion criteria provided | literature only |