Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000824492 | SCV000965391 | uncertain significance | Developmental and epileptic encephalopathy, 26 | 2018-11-21 | criteria provided, single submitter | clinical testing | In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. Experimental studies and prediction algorithms are not available for this variant, and the functional significance of the affected amino acid(s) is currently unknown. This variant has not been reported in the literature in individuals with KCNB1-related conditions. This variant is not present in population databases (ExAC no frequency). This sequence change results in a premature translational stop signal in the KCNB1 gene (p.Gln288Argfs*53). While this is not anticipated to result in nonsense mediated decay, it is expected to disrupt the last 571 amino acids of the KCNB1 protein. |