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Gene | BRAF |
Variant | G596R |
Impact List | missense |
Protein Effect | loss of function - predicted |
Gene Variant Descriptions | BRAF G596R lies within the protein kinase domain of the Braf protein (UniProt.org). G596R results in activation of Erk in the presence of CRAF (PMID: 19735675, PMID: 28783719, PMID: 18697864) and in another study demonstrates similar cell proliferation and viability levels to wild-type Braf (PMID: 29533785), but results in impaired Braf kinase activity and decreased Mek and Erk phosphorylation, including in the presence of BRAF V600E, is not transforming in culture, and does not promote tumor formation in mouse models (PMID: 19735675, PMID: 28783719), and therefore, is predicted to lead to a loss of Braf protein function. |
Associated Drug Resistance | |
Category Variants Paths |
BRAF mutant BRAF G596X BRAF G596R BRAF mutant BRAF inact mut BRAF G596R |
Transcript | NM_004333.6 |
gDNA | chr7:g.140753349C>G |
cDNA | c.1786G>C |
Protein | p.G596R |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_004333.5 | chr7:g.140753349C>G | c.1786G>C | p.G596R | RefSeq | GRCh38/hg38 |
XM_005250045 | chr7:g.140753349C>G | c.1786G>C | p.G596R | RefSeq | GRCh38/hg38 |
NM_001378468.1 | chr7:g.140753349C>G | c.1786G>C | p.G596R | RefSeq | GRCh38/hg38 |
NM_004333.6 | chr7:g.140753349C>G | c.1786G>C | p.G596R | RefSeq | GRCh38/hg38 |
NM_001378467.1 | chr7:g.140753358C>G | c.1786G>C | p.G596R | RefSeq | GRCh38/hg38 |
NM_004333 | chr7:g.140753349C>G | c.1786G>C | p.G596R | RefSeq | GRCh38/hg38 |
NM_001354609.2 | chr7:g.140753349C>G | c.1786G>C | p.G596R | RefSeq | GRCh38/hg38 |
NM_001354609.1 | chr7:g.140753349C>G | c.1786G>C | p.G596R | RefSeq | GRCh38/hg38 |
NM_001378474.1 | chr7:g.140753349C>G | c.1786G>C | p.G596R | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
BRAF G596R | colorectal cancer | sensitive | Trametinib | Preclinical - Cell culture | Actionable | In a preclinical study, Mekinist (trametinib) reduced Erk signaling and inhibited proliferation of a colorectal cancer cell line harboring BRAF G596R in culture (PMID: 28783719). | 28783719 |
BRAF G596R | colorectal cancer | resistant | Vemurafenib | Preclinical - Cell culture | Actionable | In a preclinical study, Zelboraf (vemurafenib) did not reduce activation of Erk and Mek in a colorectal cancer cell line harboring BRAF G596R in culture (PMID: 28783719). | 28783719 |
BRAF G596R | colorectal cancer | sensitive | Dabrafenib + Trametinib | Preclinical - Cell culture | Actionable | In a preclinical study, treatment with the combination of Mekinist (trametinib) and Tafinlar (dabrafenib) resulted in decreased proliferation and increased apoptosis, and enhanced ERK inhibition compared to either agent alone in a colorectal cancer cell line harboring BRAF G596R in culture (PMID: 28947956). | 28947956 |
BRAF G596R | colorectal cancer | predicted - sensitive | RMC-4550 | Preclinical - Cell culture | Actionable | In a preclinical study, RMC-4550 inhibited Erk phosphorylation and proliferation of colorectal cancer cells harboring BRAF G596R in culture (PMID: 30104724). | 30104724 |
BRAF G596R | Advanced Solid Tumor | no benefit | Vemurafenib | Clinical Study - Cohort | Actionable | In a Phase II trial (MyPathway), Zelboraf (vemurafenib) treatment resulted in an objective response in only 4% (1/23) of patient with advanced solid tumors harboring non-V600 BRAF mutations, 1 of the non-responding patients harbored BRAF G596R (PMID: 29320312; NCT02091141). | 29320312 |
BRAF G596R | lung non-small cell carcinoma | no benefit | Vemurafenib | Case Reports/Case Series | Actionable | In a Phase II trial, Zelboraf (vemurafenib) treatment did not result in response in the cohort of 15 non-small cell lung cancer patients with non-V600 BRAF mutations, which included 1 patient harboring BRAF G596R, and enrollment in this cohort was discontinued (PMID: 31959346; NCT02304809). | 31959346 |
BRAF G596R | colorectal cancer | predicted - sensitive | PLX8394 | Preclinical - Cell culture | Actionable | In a preclinical study, colorectal cancer cells harboring BRAF G596R demonstrated sensitivity to PLX8394 treatment in culture (PMID: 30559419). | 30559419 |