Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
ARUP Laboratories, |
RCV001812992 | SCV001472860 | pathogenic | not provided | 2020-08-21 | criteria provided, single submitter | clinical testing | The MSH2 c.2539_2542delAAAG; p.Lys847fs variant, to our knowledge, is not reported in the medical literature but is reported in the SITHER database (see link). This variant is absent from general population databases (Exome Variant Server, Genome Aggregation Database), indicating it is not a common polymorphism. This variant causes a frameshift by deleting 4 nucleotides, so it is predicted to result in a truncated protein or mRNA subject to nonsense-mediated decay. Based on available information, this variant is considered to be pathogenic. References Link to SITHER database for p.Lys847fs: http://www.inc.gob.ar/sither/variants/0000000040#00000004 |
Ambry Genetics | RCV002430072 | SCV002742443 | pathogenic | Hereditary cancer-predisposing syndrome | 2017-07-03 | criteria provided, single submitter | clinical testing | The c.2539_2542delAAAG pathogenic mutation, located in coding exon 15 of the MSH2 gene, results from a deletion of 4 nucleotides at nucleotide positions 2539 to 2542, causing a translational frameshift with a predicted alternate stop codon (p.K847Pfs*44). This alteration is expected to result in loss of function by premature protein truncation. As such, this alteration is interpreted as a disease-causing mutation. |