Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV004564081 | SCV000552598 | pathogenic | Familial adenomatous polyposis 1 | 2016-08-16 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. While this particular variant has not been reported in the literature, loss-of-function variants in APC are known to be pathogenic (PMID: 20685668, 17963004). In addition, multiple truncating variants downstream of this truncation have been reported as pathogenic in individuals with familial adenomatous polyposis (PMID: 17064931,  1316610 ). This sequence change deletes 2 nucleotides from exon 16 of the APC mRNA (c.2910_2911delTG), causing a frameshift at codon 970. This creates a premature translational stop signal in the last exon of the APC mRNA (p.Ser970Argfs*5). While this is not anticipated to result in nonsense mediated decay, it is expected to result in a truncated APC protein by eliminating ~1870 amino acid residues (~66%) from the full length protein. |