Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV002398311 | SCV002705978 | pathogenic | Hereditary cancer-predisposing syndrome | 2021-02-05 | criteria provided, single submitter | clinical testing | The p.E529* pathogenic mutation (also known as c.1585G>T), located in coding exon 10 of the MSH2 gene, results from a G to T substitution at nucleotide position 1585. This changes the amino acid from a glutamic acid to a stop codon within coding exon 10. This alteration was identified in 1/198 Chinese endometrial cancer patients and the tumor demonstrated loss of MSH2 and MSH6 on IHC (Tian W et al. Int J Cancer, 2019 09;145:1290-1298). 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. |