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Gene | BRAF |
Variant | A598_T599insV |
Impact List | insertion |
Protein Effect | gain of function |
Gene Variant Descriptions | BRAF A598_T599insV results in the insertion of a valine (V) in the protein kinase domain of the Braf protein between amino acids 598 and 599 (UniProt.org). A598_T599insV results in increased Braf kinase activity, increased downstream Mapk and Mek signaling, and the ability to transform cells in culture (PMID: 16501605). |
Associated Drug Resistance | |
Category Variants Paths |
BRAF mutant BRAF act mut BRAF A598_T599insV |
Transcript | NM_004333.6 |
gDNA | chr7:g.140753340_140753341insTAC |
cDNA | c.1794_1795insGTA |
Protein | p.A598_T599insV |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_004333.5 | chr7:g.140753340_140753341insTAC | c.1794_1795insGTA | p.A598_T599insV | RefSeq | GRCh38/hg38 |
NM_001354609.1 | chr7:g.140753340_140753341insTAC | c.1794_1795insGTA | p.A598_T599insV | RefSeq | GRCh38/hg38 |
NM_001378468.1 | chr7:g.140753340_140753341insTAC | c.1794_1795insGTA | p.A598_T599insV | RefSeq | GRCh38/hg38 |
NM_004333.6 | chr7:g.140753340_140753341insTAC | c.1794_1795insGTA | p.A598_T599insV | RefSeq | GRCh38/hg38 |
XM_005250045 | chr7:g.140753340_140753341insTAC | c.1794_1795insGTA | p.A598_T599insV | RefSeq | GRCh38/hg38 |
NM_004333 | chr7:g.140753340_140753341insTAC | c.1794_1795insGTA | p.A598_T599insV | RefSeq | GRCh38/hg38 |
NM_001354609.2 | chr7:g.140753340_140753341insTAC | c.1794_1795insGTA | p.A598_T599insV | RefSeq | GRCh38/hg38 |
NM_001378474.1 | chr7:g.140753340_140753341insTAC | c.1794_1795insGTA | p.A598_T599insV | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
BRAF A598_T599insV | melanoma | predicted - sensitive | Trametinib | Case Reports/Case Series | Actionable | In a Phase II trial, Mekinist (trametinib) treatment resulted in clinical benefit in six of nine melanoma patients harboring BRAF fusions or non-V600 BRAF mutations, including stable disease lasting 7.3 months with a tumor reduction of 8% in a patient harboring BRAF A598_T599insV, who also harbored KIT M541L (PMID: 33861486; NCT02296112). | 33861486 |
BRAF A598_T599insV | pilocytic astrocytoma | predicted - sensitive | Dabrafenib | Case Reports/Case Series | Actionable | In a clinical case study, Tafinlar (dabrafenib) resulted in neurological improvement and decreased tumor size after 3 months of treatment in a pediatric patient with pilocytic astrocytoma harboring BRAF A598_T599insV (reported as BRAF V599ins) (PMID: 38781546). | 38781546 |
BRAF A598_T599insV | melanoma | predicted - sensitive | Dabrafenib + Trametinib | Case Reports/Case Series | Actionable | In a clinical case study, Tafinlar (dabrafenib) and Mekinist (trametinib) combination treatment resulted in a partial response within 4 weeks with a progression-free survival of 4.6 months in a patient with metastatic melanoma harboring BRAF A598_T599insV (PMID: 28800030). | 28800030 |
BRAF A598_T599insV | melanoma | 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 limited response in the primary tumor, but regression of the abdominal lymph node and groin metastases in a patient with metastatic melanoma harboring BRAF A598_T599insV, with a decrease in the variant allele frequency of BRAF A598_T599insV (PMID: 31824282). | 31824282 |