Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003107560 | SCV003794546 | uncertain significance | Arrhythmogenic right ventricular dysplasia 10 | 2022-03-13 | criteria provided, single submitter | clinical testing | In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. This variant disrupts a region of the DSG2 protein in which other variant(s) (p.Gly678Ala) have been observed in individuals with DSG2-related conditions (PMID: 24070718). 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 DSG2-related conditions. This variant is a gross deletion of the genomic region encompassing exon(s) 14 of the DSG2 gene. This variant would be expected to be in-frame, preserving the integrity of the reading frame. |