BRAF

Chr 7AD

B-Raf proto-oncogene, serine/threonine kinase

Also known as: B-RAF1, B-raf, BRAF-1, BRAF1, NS7, RAFB1

This gene encodes a protein belonging to the RAF family of serine/threonine protein kinases. This protein plays a role in regulating the MAP kinase/ERK signaling pathway, which affects cell division, differentiation, and secretion. Mutations in this gene, most commonly the V600E mutation, are the most frequently identified cancer-causing mutations in melanoma, and have been identified in various other cancers as well, including non-Hodgkin lymphoma, colorectal cancer, thyroid carcinoma, non-small cell lung carcinoma, hairy cell leukemia and adenocarcinoma of lung. Mutations in this gene are also associated with cardiofaciocutaneous, Noonan, and Costello syndromes, which exhibit overlapping phenotypes. A pseudogene of this gene has been identified on the X chromosome. [provided by RefSeq, Aug 2017]

GeneReviewsOMIMResearchGenerating clinical summary…
GOFmechanismADLOEUF 0.217 OMIM phenotypes
VCEP Guidelines: RASopathyReleased
View SpecificationsClinGen Panel
Clinical SummaryBRAF
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Gene-Disease Validity (ClinGen)
Noonan syndrome · ADModerate

Moderate evidence — consider for supplementary testing

4 total gene-disease associations curated

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
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ClinVar Variants
129 unique Pathogenic / Likely Pathogenic· 661 VUS of 1566 total submissions
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Clinical Trials
12 active or recruiting trials — potential therapeutic options may be available
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GeneReview available — BRAF
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint?
0.21LOEUF
pLI 1.000
Z-score 5.91
OE 0.10 (0.050.21)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint?
3.72Z-score
OE missense 0.49 (0.430.55)
203 obs / 416.7 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios?
LoF OE?0.10 (0.050.21)
00.351.4
Missense OE?0.49 (0.430.55)
00.61.4
Synonymous OE?1.02
01.21.6
LoF obs/exp: 5 / 50.2Missense obs/exp: 203 / 416.7Syn Z: -0.22
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveBRAF-related Noonan syndromeGOFAD
definitiveBRAF-related LEOPARD syndromeGOFAD
definitiveBRAF-related cardiofaciocutaneous syndromeGOFAD

This gene — mechanism propensity

DN
0.5279th %ile
GOF
0.6346th %ile
LOF
0.71top 10%

This gene has evidence for multiple mechanisms of pathogenicity (loss-of-function and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to loss-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

LOFprediction above median · LOEUF 0.21
GOF1 literature citation · 98% of P/LP are missense

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Literature Evidence

GOFThe most common activating mutation present within the BRAF oncogene is associated with valine substitution for glutamate at position 600 (V600E) within the BRAF kinase.1

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

References

  1. 1.PMID 29595366

ClinVar Variant Classifications

1566 submitted variants in ClinVar

Classification Summary

Pathogenic60
Likely Pathogenic69
VUS661
Likely Benign564
Benign104
Conflicting70
60
Pathogenic
69
Likely Pathogenic
661
VUS
564
Likely Benign
104
Benign
70
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
2
58
0
0
60
Likely Pathogenic
0
69
0
0
69
VUS
50
465
129
17
661
Likely Benign
2
9
275
278
564
Benign
0
4
88
12
104
Conflicting
70
Total546054923071,528

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

47 pathogenic / likely-pathogenic (of 57) ClinVar copy-number / structural variants overlap BRAF — these span large chromosomal regions, not the gene specifically, and are excluded from the counts above. Explore in CNV tools →

Protein Context — Lollipop Plot

BRAF · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

Non Small Cell Lung CancerSmall Cell Lung CarcinomaNUT Carcinoma

Biology of Young Lung Cancer Study: The YOUNG LUNG Study

RECRUITING
NCT05265429Dana-Farber Cancer InstituteStarted 2023-01-01
Data and Specimen Collection
Carcinoma, Non-Small-Cell Lung

A Study of Participants in China With Non-Small-Cell Lung Cancer That is Unable to be Treated With Surgery

ACTIVE NOT RECRUITING
NCT05872763Hoffmann-La RocheStarted 2023-08-11
Papillary Thyroid CarcinomaThyroid NeoplasmsRET Proto-Oncogene Mutation

RET-US Study - Ultrasound-Based Prediction of RET Alterations and Lateral-Neck Metastasis in Thyroid Cancer

NOT YET RECRUITING
NCT07042984Fujian Medical UniversityStarted 2025-07-01
AI-Ultrasound RET Prediction
Advanced Thyroid Cancer Patients Who Received Target Therapy

A Retrospective and Prospective Real-world Study of Molecular Typing in the Treatment of Advanced Thyroid Cancer

RECRUITING
NCT06195228Phase PHASE4Fudan UniversityStarted 2020-01-01
dabrafenib plus trametinib with or without PD-1 antibodyentrectinib or larotrectinib with or without anti-PD-1 antibdoypralsetinib or selpercatinib with or without anti-PD-1 antibdoy
Metastatic Lung Non-Small Cell CarcinomaRecurrent Lung Non-Small Cell CarcinomaRefractory Lung Non-Small Cell Carcinoma

Atezolizumab and Cobimetinib in Treating Patients With Metastatic, Recurrent, or Refractory Non-small Cell Lung Cancer

ACTIVE NOT RECRUITING
NCT03600701Phase PHASE2National Cancer Institute (NCI)Started 2018-11-29
AtezolizumabBiopsy ProcedureBiospecimen Collection
Non-Small Cell Lung Cancer

Observational Prospective Study of Quality of Life in Unresectable TNM Stage III NSCLC (OBSTINATE)

ACTIVE NOT RECRUITING
NCT05049044Groupe Francais De Pneumo-CancerologieStarted 2020-12-18
Quality of Life Questionnaire-Core 30 (QLQ-C30)
BRAF V600 MutationLow-grade GliomaLow Grade Glioma of Brain

Dabrafenib and Trametinib for BRAF V600 Mutant Low-Grade Gliomas

RECRUITING
NCT07110246Phase PHASE2University of California, San FranciscoStarted 2025-11-07
DabrafenibTrametinibMagnetic Resonance Imaging (MRI)
Advanced CancerNon Small Cell Lung Cancer

POTENT - Tepotinib in Combination With Pembrolizumab in NSCLC

ACTIVE NOT RECRUITING
NCT05782361Phase PHASE1Institute of Cancer Research, United KingdomStarted 2023-05-03
TepotinibPembrolizumab
Non Small Cell Lung Cancer Metastatic

First-line Therapy With Nivolumab Plus Ipilimumab in Combination With Chemotherapy for Metatastatic NSCLC (NICReWo Trial)

RECRUITING
NCT07190677Fondazione IRCCS Policlinico San Matteo di PaviaStarted 2025-01-17
Thyroid Gland Anaplastic CarcinomaThyroid Gland Squamous Cell Carcinoma

Pembrolizumab, Dabrafenib, and Trametinib Before Surgery for the Treatment of BRAF-Mutated Anaplastic Thyroid Cancer

ACTIVE NOT RECRUITING
NCT04675710Phase PHASE2M.D. Anderson Cancer CenterStarted 2021-06-24
Conventional SurgeryDabrafenibIntensity-Modulated Radiation Therapy
Non Small Cell Lung CancerEGFR Gene MutationALK Gene Mutation

Treatment Strategies and Survival Outcome for Non-small Cell Lung Cancer With Oncogenic Mutation

RECRUITING
NCT04322890Phase PHASE2Hunan Province Tumor HospitalStarted 2020-04-16
OsimertinibAlectinib 150 MGCrizotinib 250 MG
Low-grade GliomaPlexiform NeurofibromaCentral Nervous System Glioma

Trametinib for Pediatric Neuro-oncology Patients With Refractory Tumor and Activation of the MAPK/ERK Pathway.

ACTIVE NOT RECRUITING
NCT03363217Phase PHASE2St. Justine's HospitalStarted 2018-08-16
Trametinib