Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001008837 | SCV001168641 | pathogenic | not provided | 2016-12-30 | criteria provided, single submitter | clinical testing | The c.34delG variant in the MED13L gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. It was not observed in the large population cohort in the Exome Aggregation Consortium (ExAC). The c.34delG variant causes a frameshift starting with codon Alanine 12, changes this amino acid to an Arginine residue, and creates a premature Stop codon at position 35 of the new reading frame, denoted p.Ala12ArgfsX35. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. Therefore, the presence of this pathogenic variant is consistent with a diagnosis of an MED13L-related disorder. |