Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
New York Genome Center | RCV002227615 | SCV002506663 | uncertain significance | Intellectual disability, autosomal dominant 6; Developmental and epileptic encephalopathy, 27 | 2021-06-25 | criteria provided, single submitter | clinical testing | The inherited c.4406G>A, p.Ser1469Asn variant identified in the GRIN2B gene has not been reported in the literature in individuals with GRIN2B-related neurodevelopmental disorders. This variant is absent in gnomAD v3.1.1, suggesting it is not a common benign variant in the populations represented in this database. In silico algorithms predict a conflicting interpretation of pathogenicity. The variant resides at N-methyl D-aspartate receptor 2B3 C-terminus domainof the GluN2B protein. This domain is found at the C-terminus of many NMDA-receptor proteins (PMID:8428958). Given the lack of compelling evidence for its pathogenicity, the inherited c.4406G>A, p.Ser1469Asn variant identified in the GRIN2B gene is reported as a Variant of UncertainSignificance. |