Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000498318 | SCV000590486 | likely pathogenic | not provided | 2017-06-14 | criteria provided, single submitter | clinical testing | A variant that is likely pathogenic has been identified in the CACNA1A gene. The c.5842+1 G>T variant has not been published as a pathogenic variant, nor has it been reported as a benign variant to our knowledge. The c.5842+1 G>T variant is not observed in large population cohorts (Lek et al., 2016; 1000 Genomes Consortium et al., 2015; Exome Variant Server). The c.5842+1 G>T splice site variant destroys the canonical splice donor site in intron 39. It is predicted to cause abnormal gene splicing, either leading to an abnormal message that is subject to nonsense-mediated mRNA decay, or to an abnormal protein product if the message is used for protein translation. Therefore, this variant is likely pathogenic; however, the possibility that it is benign cannot be excluded. |