Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001313707 | SCV001504211 | uncertain significance | Asphyxiating thoracic dystrophy 5; Senior-Loken syndrome 8 | 2021-08-30 | criteria provided, single submitter | clinical testing | This sequence change replaces phenylalanine with valine at codon 1017 of the WDR19 protein (p.Phe1017Val). The phenylalanine residue is highly conserved and there is a small physicochemical difference between phenylalanine and valine. This variant is present in population databases (rs778689889, ExAC 0.003%). This variant has not been reported in the literature in individuals affected with WDR19-related conditions. Algorithms developed to predict the effect of missense changes on protein structure and function are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Probably Damaging"; Align-GVGD: "Class C15"). In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
Broad Center for Mendelian Genomics, |
RCV001724291 | SCV001950430 | uncertain significance | Retinitis pigmentosa | 2021-04-01 | criteria provided, single submitter | curation | The p.Phe1017Val variant in WDR19 was identified in an individual with Retinitis pigmentosa, via a collaborative study between the Broad Institute's Center for Mendelian Genomics and the Pierce lab (https://oculargenomics.meei.harvard.edu/labs/pierce-lab/lab-members/). Through a review of available evidence we were able to apply the following criteria: PM2, PP3. Based on this evidence we have classified this variant as a Variant of Uncertain Significance. If you have any questions about the classification please reach out to the Pierce Lab. |
Fulgent Genetics, |
RCV002486223 | SCV002784314 | uncertain significance | Asphyxiating thoracic dystrophy 5; Nephronophthisis 13; Cranioectodermal dysplasia 4; Senior-Loken syndrome 8; Spermatogenic failure 72 | 2022-04-10 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV002543630 | SCV003663899 | uncertain significance | Inborn genetic diseases | 2022-11-18 | criteria provided, single submitter | clinical testing | The c.3049T>G (p.F1017V) alteration is located in exon 27 (coding exon 27) of the WDR19 gene. This alteration results from a T to G substitution at nucleotide position 3049, causing the phenylalanine (F) at amino acid position 1017 to be replaced by a valine (V). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |