Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Sema4, |
RCV002255945 | SCV002532292 | pathogenic | Hereditary cancer-predisposing syndrome | 2022-02-05 | criteria provided, single submitter | curation | |
Baylor Genetics | RCV003464415 | SCV004198953 | pathogenic | Familial adenomatous polyposis 2 | 2023-05-14 | criteria provided, single submitter | clinical testing | |
Color Diagnostics, |
RCV002255945 | SCV004358590 | pathogenic | Hereditary cancer-predisposing syndrome | 2023-04-03 | criteria provided, single submitter | clinical testing | This variant changes 1 nucleotide in exon 6 of the MUTYH gene, creating a premature translation stop signal. This variant is expected to result in an absent or non-functional protein product. This variant has been reported in individuals affected with colorectal cancer (PMID: 28127763, 30151276), ovarian cancer (PMID: 29371908), and pancreatic neuroendocrine tumor (PMID: 33230973). A different variant resulting in the same amino acid change, c.467G>A (p.Trp156Ter), has been reported in individual with MUTYH-associated polyposis (PMID: 34259353), colorectal cancer (with a pathogenic co-variant in APC; PMID: 25151137), neuroblastoma (PMID: 34308366), and endometrial cancer (PMID: 30886832). This variant has not been identified in the general population by the Genome Aggregation Database (gnomAD). Loss of MUTYH function is a known mechanism of disease (clinicalgenome.org). Based on the available evidence, this variant is classified as Pathogenic. |
Ambry Genetics | RCV002255945 | SCV005038304 | pathogenic | Hereditary cancer-predisposing syndrome | 2024-03-05 | criteria provided, single submitter | clinical testing | The p.W156* pathogenic mutation (also known as c.468G>A), located in coding exon 6 of the MUTYH gene, results from a G to A substitution at nucleotide position 468. This changes the amino acid from a tryptophan to a stop codon within coding exon 6. 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. |