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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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FBXO3
F-box protein 3
Chromosome 11 · 11p13
NCBI Gene: 26273Ensembl: ENSG00000110429.14HGNC: HGNC:13582UniProt: Q49AF1
82PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingnucleusresponse to lipopolysaccharideubiquitin-like ligase-substrate adaptor activitysmoking initiationplacenta praeviabreast cancerneoplasm
✦AI Summary

FBXO3 (F-box protein 3) is a substrate-recognition component of SCF-type E3 ubiquitin ligase complexes that regulates protein degradation through the ubiquitin-proteasome system 1. Primary function: FBXO3 assembles into SCFFBXO3 complexes via F-box domain interactions with SKP1 and CUL1 2, enabling recognition and ubiquitination of target proteins for proteasomal degradation. Mechanism: During viral infection, FBXO3 associates with Rift Valley fever virus NSs protein to form a remodeled filamentous E3 ligase that targets the TFIIH complex, inhibiting antiviral immunity 3. In cellular homeostasis, TBK1-dependent phosphorylation of FBXO3 facilitates TMEM192 ubiquitination to activate lysophagy, a process eliminating damaged lysosomes 45. Disease relevance: FBXO3 upregulation contributes to neuroinflammation in cerebral ischemia/reperfusion injury through HIPK2 degradation 6. Elevated FBXO3 expression correlates with poor outcomes in rheumatoid arthritis, leukemia, and oral squamous cell carcinoma 1. In breast cancer, FBXO3 stabilizes USP4 to promote PI3K-mediated metastasis 7. Clinical significance: FBXO3 represents a therapeutic target for inflammatory diseases, ischemic stroke, and malignancies, with potential for small-molecule inhibition to modulate disease progression.

Sources cited
1
FBXO3 associates with Rift Valley fever virus NSs to form a remodeled E3 ligase targeting TFIIH complex degradation
PMID: 39366381
2
FBXO3 is a critical lysophagy regulator activated by TBK1 phosphorylation to ubiquitinate TMEM192
PMID: 39875384
3
FBXO3 elevation aggravates cerebral ischemia/reperfusion injury through inflammatory response and HIPK2 degradation
PMID: 36362432
4
FBXO3 has multiple biological functions in immune regulation and is elevated in rheumatoid arthritis, leukemia, and cancers
PMID: 35127719
5
FBXO3 ApaG domain structure is required for substrate interaction and degradation
PMID: 27010866
6
FBXO3 stabilizes USP4 to promote PI3K-mediated breast cancer metastasis and correlates with poor patient survival
PMID: 38134227
7
TBK1-phosphorylated FBXO3 orchestrates lysophagic flux through TMEM192 ubiquitination and TAX1BP1 recognition
PMID: 40083080
8
Cryo-EM structure of SCFFBXO3 reveals F-box domain interactions with SKP1 and CUL1 for E3 ligase assembly
PMID: 39921442
Disease Associationsⓘ20
smoking initiationOpen Targets
0.35Weak
placenta praeviaOpen Targets
0.10Weak
breast cancerOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.08Suggestive
Ischemic strokeOpen Targets
0.02Suggestive
rheumatoid arthritisOpen Targets
0.01Suggestive
infectionOpen Targets
0.01Suggestive
disseminated candidiasisOpen Targets
0.01Suggestive
ovarian carcinomaOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
amyotrophic lateral sclerosisOpen Targets
0.01Suggestive
strokeOpen Targets
0.01Suggestive
atherosclerosisOpen Targets
0.01Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.01Suggestive
Cerebral ischemiaOpen Targets
0.01Suggestive
acute lung injuryOpen Targets
0.01Suggestive
SepsisOpen Targets
0.01Suggestive
Huntington diseaseOpen Targets
0.01Suggestive
neuroblastomaOpen Targets
0.01Suggestive
Duchenne muscular dystrophyOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
KIAA1549LProtein interaction87%SKP1Protein interaction82%CUL1Protein interaction82%FBXL2Protein interaction75%FBXL6Shared pathway50%FBXW2Shared pathway33%
Tissue Expression6 tissues
Bone Marrow
100%
Heart
91%
Brain
48%
Ovary
45%
Liver
44%
Lung
34%
Gene Interaction Network
Click a node to explore
FBXO3KIAA1549LSKP1CUL1FBXL2FBXL6FBXW2
PROTEIN STRUCTURE
Preparing viewer…
PDB5HDW · 2.00 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.45Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.28 [0.18–0.45]
RankingsWhere FBXO3 stands among ~20K protein-coding genes
  • #5,790of 20,598
    Most Researched82
  • #2,513of 17,882
    Most Constrained (LOEUF)0.45 · top quartile
Genes detectedFBXO3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Rift Valley fever virus coordinates the assembly of a programmable E3 ligase to promote viral replication.
PMID: 39366381
Cell · 2024
1.00
2
Activation of lysophagy by a TBK1-SCF
PMID: 39875384
Nat Commun · 2025
0.90
3
E3 Ubiquitin Ligase FBXO3 Drives Neuroinflammation to Aggravate Cerebral Ischemia/Reperfusion Injury.
PMID: 36362432
Int J Mol Sci · 2022
0.80
4
Diverse Roles of F-BoxProtein3 in Regulation of Various Cellular Functions.
PMID: 35127719
Front Cell Dev Biol · 2021
0.70
5
Crystal structure and interaction studies of the human FBxo3 ApaG domain.
PMID: 27010866
FEBS J · 2016
0.60