Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV004723735 | SCV005329523 | uncertain significance | Developmental and epileptic encephalopathy, 42 | 2023-05-20 | criteria provided, single submitter | clinical testing | The observed missense c.7133A>C(p.Glu2378Ala) variant in CACNA1A gene has not been reported previously as a pathogenic variant nor a benign variant, to our knowledge. The p.Glu2378Ala variant is absent in gnomAD Exomes. This variant has not been submitted to the ClinVar database. Computational evidences (SIFT - Tolerated and MutationTaster - Disease causing) predict conflicting evidence on protein structure and function for this variant. The amino acid change p.Glu2378Ala in CACNA1A is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. The amino acid Glu at position 2378 is changed to a Ala changing protein sequence and it might alter its composition and physico-chemical properties. For these reasons, this variant has been classified as a Variant of Uncertain Significance (VUS). |