Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001009280 | SCV001169102 | pathogenic | not provided | 2019-03-18 | criteria provided, single submitter | clinical testing | The c.1716_1717insC variant in the SETD5 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The c.1716_1717insC variant causes a frameshift starting with codon Asparagine 573, changes this amino acid to a Glutamine residue, and creates a premature Stop codon at position 40 of the new reading frame, denoted p.Asn573GlnfsX40. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. The c.1716_1717insC variant is not observed in large population cohorts (Lek et al., 2016). We interpret c.1716_1717insC as a pathogenic variant. |