Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
NIHR Bioresource Rare Diseases, |
RCV000851763 | SCV000899650 | likely pathogenic | Factor V deficiency | 2019-02-01 | criteria provided, single submitter | research | |
Labcorp Genetics |
RCV003596549 | SCV004293800 | pathogenic | Congenital factor V deficiency | 2023-04-12 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Ser955Alafs*4) in the F5 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in F5 are known to be pathogenic (PMID: 30924984). This variant is present in population databases (rs765982916, gnomAD 0.03%). This premature translational stop signal has been observed in individual(s) with autosomal recessive factor V deficiency (PMID: 11781258, 30924984). In at least one individual the data is consistent with being in trans (on the opposite chromosome) from a pathogenic variant. This variant is also known as 2952delT. ClinVar contains an entry for this variant (Variation ID: 627051). For these reasons, this variant has been classified as Pathogenic. |
OMIM | RCV000851763 | SCV000020836 | pathogenic | Factor V deficiency | 2002-01-15 | no assertion criteria provided | literature only |