Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000599466 | SCV000710278 | pathogenic | not provided | 2017-12-15 | criteria provided, single submitter | clinical testing | The c.1314_1315delAG variant in the KCNB1 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The c.1314_1315delAG variant causes a frameshift starting with codon Arginine 438, changes this amino acid to a Serine residue, and creates a premature Stop codon at position 21 of the new reading frame, denoted p.Arg438SerfsX21. This variant is predicted to cause loss of normal protein function through protein truncation. Thec.1314_1315delAG variant is not observed in large population cohorts (Lek et al., 2016). Weinterpret c.1314_1315delAG as a pathogenic variant. |