Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001312469 | SCV001502925 | uncertain significance | Seizures, benign familial infantile, 3; Developmental and epileptic encephalopathy, 11 | 2021-08-27 | criteria provided, single submitter | clinical testing | |
Pittsburgh Clinical Genomics Laboratory, |
RCV004783951 | SCV005397153 | uncertain significance | Episodic ataxia, type 9 | 2023-03-20 | criteria provided, single submitter | clinical testing | This sequence variant is a single nucleotide substitution (G>C) at coding position 787 in the SCN2A gene which results in an alanine to proline amino acid change at residue 263 in the SCN2A protein. This novel variant has not been reported previously in individuals with SCN2A-related disease, to our knowledge. This variant is absent from the gnomAD population database (0/~282000 alleles). Alanine is highly conserved at this protein position in vertebrates, and multiple bioinformatic tools predict that this amino acid change is deleterious. Functiol studies assessing the effect of this variant on protein structure or activity have not been performed, to our knowledge. At this time, there is insufficient evidence to determine if this variant is pathogenic or benign. Therefore, we consider it to be a variant of uncertain significance. ACMG Criteria: PM2, PP2, PP3 |