Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000481751 | SCV000573826 | pathogenic | not provided | 2017-02-24 | criteria provided, single submitter | clinical testing | The c.566_567dupGA variant in the MEF2C gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The c.566_567dupGA variant causes a frameshift starting with codon Proline 190, changes this amino acid to an Aspartic Acid residue, and creates a premature Stop codon at position 11 of the new reading frame, denoted p.Pro190AspfsX11. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. The c.566_567dupGA variant is not observed in large population cohorts (Lek et al., 2016; 1000 Genomes Consortium et al., 2015; Exome Variant Server). We interpret c.566_567dupGA as a pathogenic variant. |