Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001370450 | SCV001566939 | uncertain significance | Familial acute necrotizing encephalopathy | 2022-12-02 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with glutamine, which is neutral and polar, at codon 3079 of the RANBP2 protein (p.Arg3079Gln). 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. Algorithms developed to predict the effect of missense changes on protein structure and function (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be disruptive. ClinVar contains an entry for this variant (Variation ID: 1060949). This variant has not been reported in the literature in individuals affected with RANBP2-related conditions. This variant is present in population databases (rs532940205, gnomAD 0.003%). |
Ambry Genetics | RCV004656576 | SCV005156734 | uncertain significance | not specified | 2024-03-28 | criteria provided, single submitter | clinical testing | The c.9236G>A (p.R3079Q) alteration is located in exon 28 (coding exon 28) of the RANBP2 gene. This alteration results from a G to A substitution at nucleotide position 9236, causing the arginine (R) at amino acid position 3079 to be replaced by a glutamine (Q). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |