Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
NIHR Bioresource Rare Diseases, |
RCV000851700 | SCV000899516 | pathogenic | Hereditary factor IX deficiency disease | 2019-02-01 | criteria provided, single submitter | research | |
ARUP Laboratories, |
RCV000010930 | SCV001159356 | pathogenic | Hereditary factor VIII deficiency disease | 2019-12-03 | criteria provided, single submitter | clinical testing | The F8 c.1492G>A; p.Gly498Arg variant (rs137852414), also known as Gly479Arg, is reported in the literature in several individuals affected with hemophilia A, with a severity ranging from mild to severe (see F8 variant database and references therein). This variant is reported in ClinVar (Variation ID: 10217), but is absent from the general population databases (Exome Variant Server, Genome Aggregation Database), indicating it is not a common polymorphism. The glycine at residue 498 is highly conserved, and computational algorithms (SIFT, PolyPhen-2) predict that this is deleterious. Based on available information, this variant is considered to be pathogenic. References: Link to F8 variant database for p.Gly498Arg: http://www.factorviii-db.org/advance_search_results.php?dosearch=1&nucleotide=1492 |
OMIM | RCV000010930 | SCV000031157 | pathogenic | Hereditary factor VIII deficiency disease | 1995-01-01 | no assertion criteria provided | literature only |