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Gene | BRAF |
Variant | V600D |
Impact List | missense |
Protein Effect | gain of function |
Gene Variant Descriptions | BRAF V600D (previously reported as V599) lies within the activation segment of the kinase domain of the Braf protein (PMID: 15035987). V600D confers a gain of function to the Braf protein as demonstrated by increased kinase activity, downstream signaling, and the ability to transform cells in vitro (PMID: 15035987). |
Associated Drug Resistance | |
Category Variants Paths |
BRAF mutant BRAF act mut BRAF V600D BRAF mutant BRAF V600X BRAF V600D |
Transcript | NM_004333.6 |
gDNA | chr7:g.140753335_140753336delTGinsAC |
cDNA | c.1799_1800delTGinsAC |
Protein | p.V600D |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_001378468.1 | chr7:g.140753335_140753336delTGinsAC | c.1799_1800delTGinsAC | p.V600D | RefSeq | GRCh38/hg38 |
NM_004333.6 | chr7:g.140753335_140753336delTGinsAC | c.1799_1800delTGinsAC | p.V600D | RefSeq | GRCh38/hg38 |
XM_005250045 | chr7:g.140753335_140753336delCAinsGT | c.1799_1800delTGinsAC | p.V600D | RefSeq | GRCh38/hg38 |
NM_004333.5 | chr7:g.140753335_140753336delTGinsAC | c.1799_1800delTGinsAC | p.V600D | RefSeq | GRCh38/hg38 |
NM_004333 | chr7:g.140753335_140753336delCAinsGT | c.1799_1800delTGinsAC | p.V600D | RefSeq | GRCh38/hg38 |
NM_001378474.1 | chr7:g.140753335_140753336delTGinsAC | c.1799_1800delTGinsAC | p.V600D | RefSeq | GRCh38/hg38 |
NM_001354609.1 | chr7:g.140753335_140753336delTGinsAC | c.1799_1800delTGinsAC | p.V600D | RefSeq | GRCh38/hg38 |
NM_001354609.2 | chr7:g.140753335_140753336delTGinsAC | c.1799_1800delTGinsAC | p.V600D | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
BRAF V600D | melanoma | sensitive | CCT196969 | Preclinical | Actionable | In a preclinical study, CCT196969 inhibited MEK and ERK phosphorylation and growth of melanoma cells harboring BRAF V600D in culture (PMID: 25500121, PMID: 17210691). | 25500121 17210691 |
BRAF V600D | melanoma | sensitive | CCT241161 | Preclinical | Actionable | In a preclinical study, CCT241161 inhibited MEK and ERK phosphorylation and growth of melanoma cells harboring BRAF V600D in culture (PMID: 25500121, PMID: 17210691). | 25500121 17210691 |
BRAF V600D | melanoma | sensitive | AZ628 | Preclinical - Cell culture | Actionable | In a preclinical study, AZ628 inhibited proliferation of melanoma cell lines harboring BRAF V600D in culture (PMID: 27523909). | 27523909 |
BRAF V600D | melanoma | sensitive | TAK-632 | Preclinical - Cell culture | Actionable | In a preclinical study, TAK-632 inhibited proliferation of melanoma cell lines harboring BRAF V600D in culture (PMID: 27523909). | 27523909 |
BRAF V600D | melanoma | sensitive | PLX7904 | Preclinical - Cell culture | Actionable | In a preclinical study, PLX7904 inhibited proliferation of melanoma cell lines harboring BRAF V600D in culture (PMID: 27523909). | 27523909 |
BRAF V600D | melanoma | sensitive | Vemurafenib | Preclinical - Cell culture | Actionable | In a preclinical study, Zelboraf (vemurafenib) inhibited proliferation of melanoma cells harboring BRAF V600D in culture (PMID: 27523909). | 27523909 |
BRAF V600D | melanoma | sensitive | Dabrafenib | Preclinical - Cell culture | Actionable | In a preclinical study, Tafinlar (dabrafenib) inhibited proliferation of melanoma cells harboring BRAF V600D in culture (PMID: 27523909). | 27523909 |
BRAF V600D | pilocytic astrocytoma | predicted - sensitive | Dabrafenib + Trametinib | Case Reports/Case Series | Actionable | In a clinical case study, single Tafinlar (dabrafenib) and subsequent Tafinlar (dabrafenib) and Mekinist (trametinib) combination treatment resulted in disease stablization in a patient with pilocytic astrocytoma harboring BRAF V600D (PMID: 28784858). | 28784858 |
BRAF V600D | lung non-small cell carcinoma | predicted - sensitive | Vemurafenib | Case Reports/Case Series | Actionable | In a Phase II trial, Zelboraf (vemurafenib) treatment resulted in a objective response rate of 44.8% (43/96), median duration of response of 6.4 months, median progression-free survival (PFS) of 5.2 months, and median overall survival of 10 months in non-small cell lung cancer patients with BRAF V600 mutations, with a PFS of 3.8 months in the patient harboring BRAF V600D (PMID: 31959346; NCT02304809). | 31959346 |
BRAF V600D | melanoma | sensitive | Belvarafenib + Cobimetinib | Preclinical - Cell culture | Actionable | In a preclinical study, the addition of Cotellic (cobimetinib) to treatment with Belvarafenib (HM95573) in a melanoma cell line harboring BRAF V600D resulted in greater inhibition of cell proliferation in culture compared to single agent alone (PMID: 33953400). | 33953400 |
BRAF V600D | melanoma | sensitive | ASTX029 | Preclinical - Cell culture | Actionable | In a preclinical study, ASTX029 treatment inhibited growth of a melanoma cell line harboring BRAF V600D in culture (PMID: 34330842). | 34330842 |
BRAF V600D | ganglioglioma | predicted - sensitive | Dabrafenib + Trametinib | Case Reports/Case Series | Actionable | In a clinical case study, treatment with the combination of Tafinlar (dabrafenib) and Mekinist (trametinib) resulted in a partial response in a pediatric patient with ganglioglioma harboring BRAF V600D (PMID: 38844796; NCT03336931). | 38844796 |