Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV002409701 | SCV002674881 | likely pathogenic | Cardiovascular phenotype | 2022-02-03 | criteria provided, single submitter | clinical testing | The p.R259* variant (also known as c.775A>T), located in coding exon 3 of the APOA5 gene, results from an A to T substitution at nucleotide position 775. This changes the amino acid from an arginine to a stop codon within coding exon 3. This alteration occurs at the 3’ terminus of the APOA5 gene and is not expected to trigger nonsense-mediated mRNA decay. However, premature stop codons are typically deleterious in nature, and this variant results in the loss of approximately 30% of the protein, including a C-terminal domain that has been implicated in lipid binding (Sun G et al. Chem Phys Lipids, 2006 Sep;143:22-8). Based on the majority of available evidence to date, this variant is likely to be pathogenic. |