Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
NIHR Bioresource Rare Diseases, |
RCV000852244 | SCV000899983 | likely pathogenic | Factor V deficiency | 2019-02-01 | criteria provided, single submitter | research | |
ISTH- |
RCV000852244 | SCV002569293 | likely pathogenic | Factor V deficiency | criteria provided, single submitter | clinical testing | ||
Labcorp Genetics |
RCV003596552 | SCV004293807 | likely pathogenic | Congenital factor V deficiency | 2023-07-28 | criteria provided, single submitter | clinical testing | In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. 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 F5 protein function. ClinVar contains an entry for this variant (Variation ID: 627404). This variant is also known as p.Gly276Glu. This missense change has been observed in individual(s) with autosomal recessive factor V deficiency (PMID: 19486170, 31064749, 32833806, 34280927, 37150682). In at least one individual the data is consistent with being in trans (on the opposite chromosome) from a pathogenic variant. This variant is present in population databases (no rsID available, gnomAD 0.006%). This sequence change replaces glycine, which is neutral and non-polar, with glutamic acid, which is acidic and polar, at codon 304 of the F5 protein (p.Gly304Glu). |
NIHR Bioresource Rare Diseases, |
RCV000852245 | SCV000899984 | uncertain significance | Thromboembolism | 2019-02-01 | flagged submission | research |