Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000413159 | SCV000491526 | likely pathogenic | not provided | 2016-06-27 | criteria provided, single submitter | clinical testing | The E137K variant in the CACNA1A gene has not been reported previously as a pathogenic variant, nor as a benign variant, to our knowledge. It was not observed in approximately 6500 individuals of European and African American ancestry in the NHLBI Exome Sequencing Project, indicating it is not a common benign variant in these populations. The E137K variant is a non-conservative amino acid substitution that alters a conserved position within the S2 helical transmembrane segment of the first homologous domain. In silico analysis predicts this variant is probably damaging to the protein structure/function. The E137K variant is a strong candidate for a pathogenic variant, however the possibility it may be a rare benign variant cannot be excluded. |