Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Mendelics | RCV002249948 | SCV002519647 | pathogenic | Thrombophilia, X-linked, due to factor 8 defect | 2022-05-04 | criteria provided, single submitter | clinical testing | |
Neuberg Centre For Genomic Medicine, |
RCV004577012 | SCV005061240 | uncertain significance | Hereditary factor VIII deficiency disease | criteria provided, single submitter | clinical testing | The missense variant c.6951C>G(p.Asp2317Glu) in F8 gene has been reported in patients affected with mild hemophilia A and F8 gene related disorders (Nair et. al., 2016; Zheng et. al., 2015; Nair et. al., 2014). The observed variant has allele frequency of 0.01% in gnomAD exomes database. This variant has been submitted to the ClinVar database as pathogenic but no details available for independent assessment. The amino acid Asp at position 2317 is changed to a Glu changing protein sequence and it might alter its composition and physico-chemical properties. Functional studies will be required to confirm the pathogenicity of the variant. For these reasons, this variant has been classified as Uncertain SIgnificance (VUS). |