Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
New York Genome Center | RCV003336046 | SCV004046563 | uncertain significance | Inherited obesity | 2022-10-26 | criteria provided, single submitter | clinical testing | The c.211A>G, p.(Arg71Gly) variant identified in the NR0B2 gene substitutes a moderately conserved Arginine for Glycine at amino acid 71/258 (exon 1/2). This variant is found with low frequency in population databases (gnomAD, BRAVO-TOPMed, All of Us) with highest allele frequency of 9.1e-6 (All ofUs) suggesting it is not a common benign variant in the populations represented in those databases. In silico algorithms predict this variant to be Pathogenic (REVEL=0.719) to the function of the canonical transcript. This variant is absent from ClinVar and to our current knowledge has not been reported in affected individuals in the literature. Given the lack of compelling evidence for its pathogenicity, the c.211A>G, p.(Arg71Gly) variant identified in the NR0B2 gene is reported as a Variant of Uncertain Significance. |
Ambry Genetics | RCV004334149 | SCV004991773 | uncertain significance | not specified | 2024-02-05 | criteria provided, single submitter | clinical testing | The c.211A>G (p.R71G) alteration is located in exon 1 (coding exon 1) of the NR0B2 gene. This alteration results from a A to G substitution at nucleotide position 211, causing the arginine (R) at amino acid position 71 to be replaced by a glycine (G). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |
Prevention |
RCV003936747 | SCV004754988 | uncertain significance | NR0B2-related disorder | 2024-02-15 | no assertion criteria provided | clinical testing | The NR0B2 c.211A>G variant is predicted to result in the amino acid substitution p.Arg71Gly. To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.025% of alleles in individuals of African descent in gnomAD. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |