Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV000166142 | SCV000216914 | pathogenic | Hereditary cancer-predisposing syndrome | 2014-10-14 | criteria provided, single submitter | clinical testing | Lines of evidence used in support of classification: Alterations resulting in premature truncation (e.g.reading frame shift, nonsense) |
Invitae | RCV000226886 | SCV000282743 | pathogenic | Familial adenomatous polyposis 1 | 2016-03-02 | criteria provided, single submitter | clinical testing | This sequence change deletes 4 nucleotides from exon 16 of the APC mRNA (c.3471_3474delGAGA), causing a frameshift at codon 1157. This creates a premature translational stop signal (p.Glu1157Aspfs*7) and is expected to result in an absent or disrupted protein product. Truncating variants in APC are known to be pathogenic. This particular truncation has been reported in the literature in patients affected with familial adenomatous polyposis (PMID: 10440612, 16676398, 20685668, 17411426, 20223039). For these reasons, this variant has been classified as Pathogenic. |