Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001008897 | SCV001168703 | pathogenic | not provided | 2018-06-06 | criteria provided, single submitter | clinical testing | The c.9033_9040delGTATAACC variant causes a frameshift starting with codon Tyrosine 3012, changes this amino acid to a Glutamine residue, and creates a premature Stop codon at position 26 of the new reading frame, denoted p.Tyr3012GlnfsX26. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. The c.9033_9040delGTATAACC variant is not observed in large population cohorts (Lek et al., 2016). Although this pathogenic variant has not been previously reported to our knowledge, other loss-of-function variants in the DMD gene have been reported in the Human Gene Mutation Database in association with dystrophinopathy (Stenson et al., 2014). |