Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
ARUP Laboratories, |
RCV001002040 | SCV001159865 | likely pathogenic | Hereditary factor VIII deficiency disease | 2018-08-06 | criteria provided, single submitter | clinical testing | The F8 c.6296T>A; p.Ile2099Asn variant, also known as Ile2080Asn, is reported in individuals with Hemophilia A (see F8 database, Rydz 2013). This variant is absent from general population databases (1000 Genomes Project, Exome Variant Server, and Genome Aggregation Database), indicating it is not a common polymorphism. The isoleucine at codon 2099 is highly conserved, and computational analyses (SIFT, PolyPhen-2) predict this variant is deleterious. Based on available information, this variant is considered to be likely pathogenic. REFERENCES F8 Variant Database link: http://www.factorviii-db.org/advance_search_results.php Rydz N et al. The Canadian National Program for hemophilia mutation testing" database: a ten-year review. Am J Hematol. 2013 Dec;88(12):1030-4. " |