Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV000570847 | SCV000674006 | pathogenic | Hereditary cancer-predisposing syndrome | 2017-06-23 | criteria provided, single submitter | clinical testing | The p.S83* pathogenic mutation (also known as c.248C>G), located in coding exon 3 of the MUTYH gene, results from a C to G substitution at nucleotide position 248. This changes the amino acid from a serine to a stop codon within coding exon 3. 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. |
Counsyl | RCV000576504 | SCV000678200 | likely pathogenic | Familial adenomatous polyposis 2 | 2016-12-12 | criteria provided, single submitter | clinical testing | |
Gene |
RCV000657766 | SCV000779519 | likely pathogenic | not provided | 2018-04-10 | criteria provided, single submitter | clinical testing | This variant is denoted MUTYH c.248C>G at the cDNA level and p.Ser83Ter (S83X) at the protein level. The substitution creates a nonsense variant, which changes a Serine to a premature stop codon (TCA>TGA), and is predicted to cause loss of normal protein function through either protein truncation or nonsense-mediated mRNA decay. Although this variant has not, to our knowledge, been reported in the literature, it is considered likely pathogenic. |
Labcorp Genetics |
RCV000576504 | SCV000965432 | pathogenic | Familial adenomatous polyposis 2 | 2023-09-29 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 485907). This variant has not been reported in the literature in individuals affected with MUTYH-related conditions. This variant is present in population databases (rs370124822, gnomAD 0.0009%). This sequence change creates a premature translational stop signal (p.Ser83*) in the MUTYH gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in MUTYH are known to be pathogenic (PMID: 18534194, 20663686). |
Baylor Genetics | RCV000576504 | SCV004199414 | likely pathogenic | Familial adenomatous polyposis 2 | 2022-11-16 | criteria provided, single submitter | clinical testing |