Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000861890 | SCV001002304 | benign | Hereditary factor IX deficiency disease; Thrombophilia, X-linked, due to factor 9 defect | 2025-01-27 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV002495223 | SCV002810038 | likely benign | Hereditary factor IX deficiency disease; Thrombophilia, X-linked, due to factor 9 defect; Warfarin sensitivity, X-linked | 2022-04-14 | criteria provided, single submitter | clinical testing | |
Department of Pathology and Laboratory Medicine, |
RCV001358071 | SCV001553719 | benign | not specified | no assertion criteria provided | clinical testing | The F9 p.Glu323Lys variant was identified in 1 male with hemophilia B from a cohort of 3000 individuals (2900 male, 100 female) (frequency: 0.00032258) (Johnsen_ 2017_PMID:29296726). This variant was also reported in 1/3713 individuals with hemophilia B in the Factor IX Gene (F9) Variant Database. The variant was identified in dbSNP (ID: rs150351950), ClinVar (classified as benign by Invitae) and LOVD 3.0.The variant was identified in control databases in 175 of 205225 chromosomes (41 hemizygous) at a frequency of 0.0008527 increasing the likelihood this could be a low frequency benign variant (Genome Aggregation Database March 6, 2019, v2.1.1). The variant was observed in the following populations: African in 168 of 19054 chromosomes (freq: 0.008817), Latino in 6 of 28025 chromosomes (freq: 0.000214) and East Asian in 1 of 14860 chromosomes (freq: 0.000067), but was not observed in the Ashkenazi Jewish, European (Finnish), European (non-Finnish), Other, and South Asian populations. The p.Glu323 residue is not conserved in mammals and computational analyses (PolyPhen-2, SIFT, AlignGVGD, BLOSUM, MutationTaster) do not suggest a high likelihood of impact to the protein; however, this information is not predictive enough to rule out pathogenicity. The variant occurs outside of the splicing consensus sequence and in silico or computational prediction software programs (SpliceSiteFinder, MaxEntScan, NNSPLICE, GeneSplicer) do not predict a difference in splicing. In summary, based on the above information this variant meets our laboratory's criteria to be classified as benign. | |
Natera, |
RCV001830862 | SCV002082228 | likely benign | Hereditary factor IX deficiency disease | 2019-12-24 | no assertion criteria provided | clinical testing | |
Prevention |
RCV003955577 | SCV004772286 | likely benign | F9-related disorder | 2022-03-04 | no assertion criteria provided | clinical testing | This variant is classified as likely benign based on ACMG/AMP sequence variant interpretation guidelines (Richards et al. 2015 PMID: 25741868, with internal and published modifications). |