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Gene | DNMT3A |
Variant | R882H |
Impact List | missense |
Protein Effect | loss of function |
Gene Variant Descriptions | DNMT3A R882H lies within the SAM-dependent MTase C5-type domain of the Dnmt3a protein (UniProt.org). R882H disrupts the tetramerization ability of the Dnmt3a protein, resulting in reduced DNA binding affinity and altered catalytic activity in in vitro assays (PMID: 22722925), disrupts Tp53 inhibition of methylation (PMID: 31640986), and results in hypomethylation, cytokine-independent growth, increased cell proliferation and impaired differentiation in cultured cells (PMID: 31164355, PMID: 32015320). |
Associated Drug Resistance | |
Category Variants Paths |
DNMT3A mutant DNMT3A inact mut DNMT3A R882H |
Transcript | NM_022552.5 |
gDNA | chr2:g.25234373C>T |
cDNA | c.2645G>A |
Protein | p.R882H |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_022552.4 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
NM_175629 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
XM_017003526 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
XM_017003526.2 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
NM_175629.2 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
XM_017003526.1 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
NM_022552 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
XM_005264175.5 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
NM_175629.2 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
XM_005264175 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
XM_005264175.6 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
NM_022552.5 | chr2:g.25234373C>T | c.2645G>A | p.R882H | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
DNMT3A mutant | acute myeloid leukemia | not applicable | N/A | Clinical Study | Prognostic | In clinical analyses, mutations in DNMT3A were associated with poor prognosis and shorter overall survival in patients with acute myeloid leukemia (PMID: 22490330, PMID: 21881046, PMID: 21670448). | 22490330 21881046 21670448 |
DNMT3A mutant | myelofibrosis | not applicable | N/A | Guideline | Prognostic | DNMT3A mutations are associated with inferior overall survival in patients with primary myelofibrosis (NCCN.org). | detail... |
DNMT3A mutant | acute myeloid leukemia | sensitive | Pinometostat | Preclinical - Cell line xenograft | Actionable | In a preclinical study, Pinometostat (EPZ-5676) treatment of acute myeloid leukemia cell lines and xenografts resulted in apoptosis, cell-cycle arrest, and terminal differentiation (PMID: 27335278). | 27335278 |
DNMT3A mutant | acute myeloid leukemia | predicted - sensitive | Decitabine | Clinical Study - Cohort | Actionable | In a clinical study, acute myeloid leukemia patients harboring DNMT3A mutations demonstrated a greater complete response rate (60% vs 33%) compared to patients with wild-type DNMT3A when treated with frontline hypomethylating agents such as Dacogen (decitabine) (PMID: 27418649). | 27418649 |
DNMT3A mutant | angioimmunoblastic T-cell lymphoma | not applicable | N/A | Guideline | Diagnostic | DNMT3A mutations aid in the diagnosis of angioimmunoblastic T-cell lymphoma (NCCN.org). | detail... |
DNMT3A mutant | acute myeloid leukemia | resistant | Olaparib | Preclinical - Patient cell culture | Actionable | In a preclinical study, patient-derived acute myeloid leukemia cells harboring a DNMT3A mutation along with FLT3-ITD and NPM1 mutation were resistant to Lynparza (olaparib) in culture (PMID: 34215619). | 34215619 |