Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000187400 | SCV000240987 | uncertain significance | not provided | 2014-02-06 | criteria provided, single submitter | clinical testing | p.Gly127Arg (GGA>AGA): c.379 G>A in exon 2 of the EPM2A gene (NM_005670.3). The Gly127Arg missense change in the EPM2A gene has not been published as a mutation, nor has it been reported as a benign polymorphism to our knowledge. It was not observed in approximately 6,500 individuals of European and African American ancestry in the NHLBI Exome Sequencing Project, indicating it is not a common benign variant in these populations. This variant is a non-conservative amino acid substitution of an uncharged, non-polar Glycine residue with a positively charged Arginine residue. In silico analysis predicts this variant is probably damaging to the protein structure/function. However, the variant alters a position that is not conserved across species. Therefore, based on the currently available information, it is unclear whether Gly127Arg is a disease-causing mutation or a rare benign variant. The variant is found in CHILD-EPI panel(s). |