Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000825520 | SCV000966834 | likely pathogenic | Factor V deficiency | 2018-08-14 | criteria provided, single submitter | clinical testing | The p.Tyr558X variant in F5 has not been reported in individuals with Factor V d eficiency, but has been identified in 1/8732 African chromosomes by the Genome A ggregation Database (gnomAD, http://gnomad.broadinstitute.org; dbSNP rs905672088 ). This nonsense variant leads to a premature termination codon at position 558, which is predicted to lead to a truncated or absent protein. Loss of function o f the F5 gene is an established disease mechanism in autosomal recessive Factor V deficiency. In summary, although additional studies are required to fully esta blish its clinical significance, this variant is likely pathogenic. ACMG/AMP Cri teria applied: PVS1; PM2. |