Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Mendelics | RCV000991023 | SCV001142124 | pathogenic | Hereditary factor VIII deficiency disease | 2019-05-28 | criteria provided, single submitter | clinical testing | |
ARUP Laboratories, |
RCV000991023 | SCV002048170 | pathogenic | Hereditary factor VIII deficiency disease | 2021-08-26 | criteria provided, single submitter | clinical testing | The F8 c.4936delG; p.Ala1646GlnfsTer17 variant (rs1603433729) is reported in the literature in an individual affected with severe hemophilia A (Gorziza 3013). This variant is also reported in ClinVar (Variation ID: 804140). This variant is absent from the Genome Aggregation Database, indicating it is not a common polymorphism. This variant causes a frameshift by deleting a single nucleotide, so it is predicted to result in a truncated protein or mRNA subject to nonsense-mediated decay. Based on available information, this variant is considered to be pathogenic. References: Gorziza RP et al. Genetic changes in severe haemophilia A: new contribution to the aetiology of a complex disease. Blood Coagul Fibrinolysis. 2013 Mar;24(2):164-9. Erratum in: Blood Coagul Fibrinolysis. 2014 Jun;25(4):405. PMID: 23249616. |