Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Prevention |
RCV003985608 | SCV004111659 | likely pathogenic | CACNA1A-related disorder | 2023-03-29 | criteria provided, single submitter | clinical testing | The CACNA1A c.978+1G>A variant is predicted to disrupt the GT donor site and interfere with normal splicing. This variant was reported in multiple members of a multi-generational family with episodic ataxia 2 (referred to as 1253+1G>A, Subramony et al. 2003. PubMed ID: 14681882). This variant has not been reported in a large population database (http://gnomad.broadinstitute.org), indicating this variant is rare. Variants that disrupt the consensus splice donor site in CACNA1A are expected to be pathogenic. This variant is interpreted as likely pathogenic. |