Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV002443888 | SCV002736626 | pathogenic | Hereditary cancer-predisposing syndrome | 2018-05-22 | criteria provided, single submitter | clinical testing | The c.2270dupA pathogenic mutation (also known as p.Y757*), located in coding exon 14 of the MSH2 gene, results from a duplication of A at nucleotide position 2270. This changes the amino acid from a tyrosine to a stop codon within coding exon 14. This mutation (designated as Y757X) was reported in a patient with MSH2/MSH6-absent colorectal cancer diagnosed at age 28 whose family met Amsterdam criteria (Jasperson KW et al. Fam. Cancer 2010 Jun;9:99-107). In addition to the clinical data presented in the literature, this alteration is expected to result in loss of function by premature protein truncation or nonsense-mediated mRNA decay. As such, this alteration is interpreted as a disease-causing mutation. |