Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003771251 | SCV001584352 | pathogenic | Familial adenomatous polyposis 1 | 2015-01-02 | criteria provided, single submitter | clinical testing | This sequence change inserts 2 nucleotides in exon 16 of the APC mRNA (c.4591_4592dupAA), causing a frameshift at codon 1531. This creates a premature translational stop signal in the last exon of the APC mRNA (p.Asn1531Lysfs*35). While this is not anticipated to result in nonsense mediated decay, it is expected to result in a truncated APC protein lacking 1308 C-terminal amino acid residues. Truncating sequence changes in APC are known to be pathogenic. This particular truncation has been reported in the literature in a patient affected with FAP (PMID: 23159591). For these reasons, this sequence change has been classified as Pathogenic. |