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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ARAF
A-Raf proto-oncogene, serine/threonine kinase
Chromosome X · Xp11.3
NCBI Gene: 369Ensembl: ENSG00000078061.15HGNC: HGNC:646UniProt: A0A024R178
150PubMed Papers
20Diseases
3Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
KinaseOncogene
CLINICAL
FDA Approved Target
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
negative regulation of apoptotic processplasma membraneregulation of TOR signalingregulation of proteasomal ubiquitin-dependent protein catabolic processcancerCostello syndromeNoonan syndromehypertrophic cardiomyopathy
✦AI Summary

ARAF is a serine/threonine kinase and member of the RAF family that functions as a key component of the RAS-RAF-MEK-ERK mitogen-activated protein kinase (MAPK) signaling cascade 1. This pathway controls cellular growth and proliferation, and ARAF catalyzes ATP-dependent phosphorylation events that propagate signals from receptor tyrosine kinases downstream to MEK1/2 and ERK1/2 kinases 2. Mechanistically, ARAF operates within a three-layered coherently orchestrated signaling module that directs physiological functions including cell cycle progression and survival 1. ARAF mutations drive cancer through activation of MAPK signaling. Rare but recurrent activating mutations in ARAF have been identified across diverse malignancies including lung adenocarcinoma, where variants such as S214C and S214F demonstrate functional importance for tumor formation and drug resistance 3. ARAF mutations also occur in histiocytic neoplasms and Erdheim-Chester disease, where they activate ERK signaling 45. Additionally, ARAF mutations are found in Rosai-Dorfman-Destombes disease lesions, suggesting clonal disease origins 6. Clinically, ARAF-mutant tumors respond to MEK inhibition. A clinical trial demonstrated 89% overall response rate with the MEK1/2 inhibitor cobimetinib in histiocytic neoplasms regardless of genotype, including ARAF-mutant cases 4. RAF inhibitor sensitivity varies by inhibitor class, with type I inhibitors showing equipotency across RAF isoforms, while type II inhibitors exhibit relative sparing of ARAF 2.

Sources cited
1
ARAF is one of three RAF isoforms in the RAF-MEK-ERK MAPK signaling cascade that controls cell proliferation and survival; genetic alterations in this pathway are prevalent in human cancers
PMID: 33992782
2
ARAF is a serine/threonine kinase component of RAS-RAF-MEK-ERK pathway; rare activating mutations occur in diverse cancers; type II RAF inhibitors exhibit relative sparing of ARAF compared to CRAF
PMID: 41072765
3
ARAF S214C and S214F variants are impactful, functionally important rare somatic mutations identified in lung adenocarcinomas that induce tumor formation and confer resistance to EGFR inhibition
PMID: 27478040
4
MEK inhibitor cobimetinib showed 89% overall response rate in histiocytic neoplasms with ARAF mutations, demonstrating MAPK pathway dependence and ERK activation by ARAF mutations
PMID: 30867592
5
ARAF mutations are abundant somatic mutations in Erdheim-Chester disease, activating the MAPK/ERK signaling pathway
PMID: 34911328
6
ARAF mutations have been found in lesional tissues of Rosai-Dorfman-Destombes disease, raising possibility of clonal origin in some forms
PMID: 29720485
7
ARAF mutations are rare oncogenic alterations identified in non-small-cell lung cancers with actionable therapeutic implications
PMID: 36806787
Disease Associationsⓘ20
cancerOpen Targets
0.56Moderate
Costello syndromeOpen Targets
0.54Moderate
hypertrophic cardiomyopathyOpen Targets
0.54Moderate
Noonan syndromeOpen Targets
0.54Moderate
low grade gliomaOpen Targets
0.50Moderate
lymphatic malformation 12Open Targets
0.42Moderate
colorectal adenocarcinomaOpen Targets
0.28Weak
lung adenocarcinomaOpen Targets
0.24Weak
small cell lung carcinomaOpen Targets
0.21Weak
melanomaOpen Targets
0.20Weak
hepatocellular carcinomaOpen Targets
0.19Weak
non-small cell lung carcinomaOpen Targets
0.19Weak
papillary thyroid carcinomaOpen Targets
0.19Weak
urinary bladder carcinomaOpen Targets
0.19Weak
head and neck squamous cell carcinomaOpen Targets
0.19Weak
chronic lymphocytic leukemiaOpen Targets
0.19Weak
acute myeloid leukemiaOpen Targets
0.19Weak
lung carcinomaOpen Targets
0.19Weak
osteosarcomaOpen Targets
0.19Weak
pancreatic ductal adenocarcinomaOpen Targets
0.19Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Drug Targets3
BELVARAFENIBPhase II
RAF serine/threonine protein kinase inhibitor
neoplasm
LY-3009120Phase I
Serine/threonine-protein kinase A-Raf inhibitor
TOVORAFENIBApproved
RAF serine/threonine protein kinase inhibitor
low grade glioma
Related Genes
RALGDSProtein interaction100%HSP90AA1Protein interaction100%HSP90AB1Protein interaction100%KRASProtein interaction100%CDC37Protein interaction100%RAF1Protein interaction99%
Tissue Expression6 tissues
Liver
100%
Lung
99%
Bone Marrow
77%
Heart
70%
Ovary
69%
Brain
27%
Gene Interaction Network
Click a node to explore
ARAFRALGDSHSP90AA1HSP90AB1KRASCDC37RAF1
PROTEIN STRUCTURE
Preparing viewer…
PDB8VSL · 1.42 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.56Moderately Constrained
pLIⓘ
0.77Intermediate
Observed/Expected LoF0.37 [0.26–0.56]
RankingsWhere ARAF stands among ~20K protein-coding genes
  • #3,010of 20,598
    Most Researched150 · top quartile
  • #896of 1,025
    FDA-Approved Drug Targets1
  • #3,586of 17,882
    Most Constrained (LOEUF)0.56 · top quartile
Genes detectedARAF
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
RAF-MEK-ERK pathway in cancer evolution and treatment.
PMID: 33992782
Semin Cancer Biol · 2022
1.00
2
Rare molecular subtypes of lung cancer.
PMID: 36806787
Nat Rev Clin Oncol · 2023
0.90
3
Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease.
PMID: 29720485
Blood · 2018
0.80
4
High-throughput Phenotyping of Lung Cancer Somatic Mutations.
PMID: 27478040
Cancer Cell · 2016
0.70
5
Efficacy of MEK inhibition in patients with histiocytic neoplasms.
PMID: 30867592
Nature · 2019
0.60