Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000413632 | SCV000492105 | uncertain significance | not specified | 2016-11-28 | criteria provided, single submitter | clinical testing | A variant of uncertain significance has been identified in the CACNA1A gene. The R1983Q variant has not been published as a pathogenic variant, nor has it been reported as a benign variant to our knowledge. The R1983Q variant is not observed at a significant frequency in large population cohorts (Lek et al., 2016; 1000 Genomes Consortium et al., 2015; Exome Variant Server). The R1983Q variant is a semi-conservative amino acid substitution, which may impact secondary protein structure as these residues differ in some properties. This substitution occurs at a position that is conserved across species. However, in silico analysis is inconsistent in its predictions as to whether or not the variant is damaging to the protein structure/function. Therefore, based on the currently available information, it is unclear whether this variant is a pathogenic variant or a rare benign variant. |
Eurofins Ntd Llc |
RCV000727526 | SCV000709483 | uncertain significance | not provided | 2017-06-21 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000791501 | SCV000930753 | likely benign | Episodic ataxia type 2; Developmental and epileptic encephalopathy, 42 | 2023-12-23 | criteria provided, single submitter | clinical testing | |
New York Genome Center | RCV002275037 | SCV002564183 | uncertain significance | Migraine, familial hemiplegic, 1; Developmental and epileptic encephalopathy, 42 | 2021-09-26 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV002524653 | SCV003725963 | uncertain significance | Inborn genetic diseases | 2022-04-13 | criteria provided, single submitter | clinical testing | The c.5948G>A (p.R1983Q) alteration is located in exon 41 (coding exon 41) of the CACNA1A gene. This alteration results from a G to A substitution at nucleotide position 5948, causing the arginine (R) at amino acid position 1983 to be replaced by a glutamine (Q). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |