Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002521861 | SCV003440509 | uncertain significance | not provided | 2022-11-28 | criteria provided, single submitter | clinical testing | In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is expected to disrupt SERPINH1 protein function. ClinVar contains an entry for this variant (Variation ID: 265865). This missense change has been observed in individuals with clinical features of osteogenesis imperfecta (PMID: 25510505; Invitae). This variant is not present in population databases (gnomAD no frequency). This sequence change replaces methionine, which is neutral and non-polar, with threonine, which is neutral and polar, at codon 237 of the SERPINH1 protein (p.Met237Thr). |
Laboratorio de Genetica e Diagnostico Molecular, |
RCV000256253 | SCV003806808 | likely pathogenic | Osteogenesis imperfecta type 10 | 2022-09-05 | criteria provided, single submitter | clinical testing | ACMG classification criteria: PS3 supporting, PS4 moderated, PM2 moderated, PM3 moderated, PP1 supporting, PP3 supporting |
OMIM | RCV000256253 | SCV000322814 | pathogenic | Osteogenesis imperfecta type 10 | 2016-10-12 | no assertion criteria provided | literature only |