Missing content? – Request curation!
Request curation for specific Genes, Variants, or PubMed publications.
Have questions, comments, or suggestions? - Let us know!
Email us at : ckbsupport@genomenon.com
Gene | MSH6 |
Variant | T1219D |
Impact List | missense |
Protein Effect | loss of function |
Gene Variant Descriptions | MSH6 T1219D lies within the ATPase domain of the Msh6 protein (PMID: 17531815). T1219D (corresponding to T1217D in mouse) retains the ability to recognize a DNA mismatch (PMID: 22277660, PMID: 15324697), results in protein expression, stability, subcellular localization, and Msh2 stability similar to wild-type Msh6, and mediates apoptosis in response to DNA damaging agents similar to wild-type Msh6 in cultured cells (PMID: 15324697) but results in loss of mismatch repair activity (PMID: 22277660, PMID: 15324697), acts as a dominant negative in in vitro assays (PMID: 22277660), and increases microsatellite instability in mouse cells (PMID: 15324697). |
Associated Drug Resistance | |
Category Variants Paths |
MSH6 mutant MSH6 inact mut MSH6 T1219D |
Transcript | NM_000179.3 |
gDNA | chr2:g.47806212_47806213delACinsGA |
cDNA | c.3655_3656delACinsGA |
Protein | p.T1219D |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_001406809.1 | chr2:g.47806212_47806213delACinsGA | c.3655_3656delACinsGA | p.T1219D | RefSeq | GRCh38/hg38 |
NM_001406796.1 | chr2:g.47806212_47806213delACinsGA | c.3655_3656delACinsGA | p.T1219D | RefSeq | GRCh38/hg38 |
NM_000179.2 | chr2:g.47806212_47806213delACinsGA | c.3655_3656delACinsGA | p.T1219D | RefSeq | GRCh38/hg38 |
XM_024452819.1 | chr2:g.47806212_47806213delACinsGA | c.3655_3656delACinsGA | p.T1219D | RefSeq | GRCh38/hg38 |
NM_000179.3 | chr2:g.47806212_47806213delACinsGA | c.3655_3656delACinsGA | p.T1219D | RefSeq | GRCh38/hg38 |
NM_001406808.1 | chr2:g.47806212_47806213delACinsGA | c.3655_3656delACinsGA | p.T1219D | RefSeq | GRCh38/hg38 |
NM_001406800.1 | chr2:g.47806212_47806213delACinsGA | c.3655_3656delACinsGA | p.T1219D | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
MSH6 mutant | rectum cancer | not applicable | N/A | Guideline | Risk Factor | Lynch syndrome results from germline mutations in DNA mismatch repair genes including MLH1, MSH2, MSH6, and PMS2, and is associated with increased risk of developing colorectal cancer (NCCN.org). | detail... |
MSH6 mutant | small intestine adenocarcinoma | not applicable | N/A | Guideline | Risk Factor | Lynch syndrome results from germline mutations in DNA mismatch repair genes including MLH1, MSH2, MSH6, and PMS2, and is associated with increased risk of developing small bowel adenocarcinoma (NCCN.org). | detail... |
MSH6 mutant | endometrial carcinoma | not applicable | N/A | Guideline | Risk Factor | Germline mutations in MSH6 result in Lynch syndrome, which is associated with increased risk of developing endometrial carcinoma (NCCN.org). | detail... |
MSH6 mutant | colorectal cancer | not applicable | N/A | Clinical Study | Diagnostic | Germline mutations in MSH6 are associated with microsatellite instability in colorectal cancer (CRC), and are diagnostic for Lynch syndrome (hereditary nonpolyposis colorectal cancer) in colorectal cancer patients (PMID: 26582061; PMID: 19125127). | 26582061 19125127 |
MSH6 mutant | pancreatic cancer | not applicable | N/A | Guideline | Risk Factor | Germline mutations in MSH6 result in Lynch syndrome, which is associated with increased risk of developing pancreatic cancer (NCCN.org). | detail... |
MSH6 mutant | colon cancer | not applicable | N/A | Guideline | Risk Factor | Lynch syndrome results from germline mutations in DNA mismatch repair genes including MLH1, MSH2, MSH6, and PMS2, and is associated with increased risk of developing colon cancer (NCCN.org). | detail... |
MSH6 mutant | ovarian cancer | not applicable | N/A | Guideline | Risk Factor | Germline mutations in MSH6 result in Lynch syndrome, which is associated with increased risk of ovarian cancer (NCCN.org). | detail... |
MSH6 mutant | stomach cancer | not applicable | N/A | Guideline | Risk Factor | Germline mutations in MSH6 result in Lynch syndrome, which is associated with increased risk of developing pancreatic cancer (NCCN.org). | detail... |