Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001958578 | SCV002232726 | pathogenic | Episodic ataxia type 2; Developmental and epileptic encephalopathy, 42 | 2021-11-15 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. This variant disrupts a region of the CACNA1A protein in which other variant(s) (p.Glu147Lys) have been determined to be pathogenic (PMID: 15483044). This suggests that this is a clinically significant region of the protein, and that variants that disrupt it are likely to be disease-causing. This variant has not been reported in the literature in individuals affected with CACNA1A-related conditions. This variant is a gross deletion of the genomic region encompassing exon(s) 2-3 of the CACNA1A gene. This variant would be expected to be in-frame, preserving the integrity of the reading frame. |