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Gene | BRAF |
Variant | D594N |
Impact List | missense |
Protein Effect | loss of function - predicted |
Gene Variant Descriptions | BRAF D594N lies within the protein kinase domain of the Braf protein (UniProt.org). D594N leads to activation of Erk signaling through CRAF but results in impaired Braf kinase activity in cell culture (PMID: 28783719) and decreased transformation ability compared to wild-type Braf in one of two cell lines in culture (PMID: 29533785), and therefore, is predicted to lead to a loss of Braf protein function. |
Associated Drug Resistance | |
Category Variants Paths |
BRAF mutant BRAF D594X BRAF D594N BRAF mutant BRAF inact mut BRAF D594N |
Transcript | NM_004333.6 |
gDNA | chr7:g.140753355C>T |
cDNA | c.1780G>A |
Protein | p.D594N |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_004333.5 | chr7:g.140753355C>T | c.1780G>A | p.D594N | RefSeq | GRCh38/hg38 |
NM_001354609.2 | chr7:g.140753355C>T | c.1780G>A | p.D594N | RefSeq | GRCh38/hg38 |
NM_001354609.1 | chr7:g.140753355C>T | c.1780G>A | p.D594N | RefSeq | GRCh38/hg38 |
NM_001378474.1 | chr7:g.140753355C>T | c.1780G>A | p.D594N | RefSeq | GRCh38/hg38 |
NM_001378468.1 | chr7:g.140753355C>T | c.1780G>A | p.D594N | RefSeq | GRCh38/hg38 |
NM_004333.6 | chr7:g.140753355C>T | c.1780G>A | p.D594N | RefSeq | GRCh38/hg38 |
NM_004333 | chr7:g.140753355C>T | c.1780G>A | p.D594N | RefSeq | GRCh38/hg38 |
XM_005250045 | chr7:g.140753355C>T | c.1780G>A | p.D594N | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
BRAF D594N | female reproductive organ cancer | no benefit | Trametinib | Case Reports/Case Series | Actionable | In a Phase II trial (NCI-MATCH), Mekinist (trametinib) treatment did not demonstrate clinical activity in patients with advanced solid tumors or lymphoma harboring BRAF fusion or non-V600 mutations, resulted in stable disease in a patient with gynecological cancer harboring BRAF D594N (PMID: 31924734; NCT02465060). | 31924734 |
BRAF D594N | Advanced Solid Tumor | predicted - resistant | Vemurafenib | Preclinical - Cell culture | Actionable | In a preclinical study, Zelboraf (vemurafenib) did not reduce activation of Mek in transformed cells expressing BRAF D594N in culture (PMID: 28783719). | 28783719 |
BRAF D594N | colorectal cancer | not predictive | Cetuximab | Preclinical - Pdx | Actionable | In a preclinical study, Erbitux (cetuximab) treatment inhibited tumor growth in a colorectal cancer patient-derived xenograft (PDX) model harboring BRAF D594N (PMID: 31515458). | 31515458 |
BRAF D594N | gallbladder cancer | predicted - sensitive | Binimetinib + Encorafenib | Case Reports/Case Series | Actionable | In a Phase II trial (BEAVER), combination treatment with Mektovi (binimetinib) and Braftovi (encorafenib) led to stable disease in a gallbladder cancer patient harboring BRAF D594N, with a treatment duration of 4.4 months (Annals of Oncology 32 (2021): S596; NCT03839342). | detail... |
BRAF D594N | colorectal cancer | not predictive | Irinotecan + Panitumumab | Case Reports/Case Series | Actionable | In a clinical study, the combination of Vectibix (panitumumab) with Camptosar (irinotecan) as a third-line therapy resulted in partial responses with 14.9 and 14.7 months progression-free survival in two patients with metastatic colorectal cancer harboring BRAF D594N (PMID: 31515458). | 31515458 |
BRAF D594N | lung non-small cell carcinoma | predicted - sensitive | RMC-4550 | Preclinical - Pdx | Actionable | In a preclinical study, RMC-4550 treatment resulted in decreased Erk phosphorylation and dose-dependent tumor growth inhibition in patient-derived xenograft (PDX) models of non-small cell lung cancer harboring BRAF D594N (PMID: 30104724). | 30104724 |
BRAF D594N | lung adenocarcinoma | predicted - sensitive | NST-628 | Preclinical - Pdx | Actionable | In a preclinical study, NST-628 treatment resulted in an overall response rate of 69.5% in a panel of patient-derived xenograft (PDX) models of solid tumors harboring RAS-MAPK pathway alterations, including 1 response out of 2 lung adenocarcinoma patient-derived xenograft (PDX) models harboring BRAF D594N (PMID: 38588399). | 38588399 |