Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000357233 | SCV000330475 | pathogenic | not provided | 2017-12-29 | criteria provided, single submitter | clinical testing | The c.467dupT pathogenic variant in the DCX gene causes a frameshift starting with codon Methionine 156, changes this amino acid to a Isoleucine residue and creates a premature Stop codon at position 9 of the new reading frame, denoted p.M156IfsX9. This pathogenic variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. Although this pathogenic variant has not been previously reported to our knowledge, other frameshift variants have been reported in Human Gene Mutation Database in association with DCX-related disorders (Stenson et al., 2014). |