Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000181208 | SCV000233486 | pathogenic | not provided | 2014-05-28 | criteria provided, single submitter | clinical testing | p.Gln257Stop (CAA>TAA): c.769 C>T in exon 7 of the DSG2 gene (NM_001943.3). The Q257X mutation in the DSG2 gene has not been reported as a disease-causing mutation or as a benign polymorphism to our knowledge. Q257X is predicted to cause loss of normal protein function either by protein truncation or nonsense-mediated mRNA decay. Other nonsense mutations in the DSG2 gene have been reported in association with ARVC. In summary, Q257X in the DSG2 gene is interpreted as a disease-causing mutation. The variant is found in ARVC panel(s). |
Center for Genomic Medicine, |
RCV003988832 | SCV004805336 | pathogenic | Arrhythmogenic right ventricular dysplasia 10 | 2024-03-25 | criteria provided, single submitter | research |