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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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FBF1
Fas binding factor 1
Chromosome 17 · 17q25.1
NCBI Gene: 85302Ensembl: ENSG00000188878.21HGNC: HGNC:24674UniProt: Q8TES7
41PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cilium assemblyapical junction complexkeratin filamentprotein bindinghypertensioncardiovascular diseaseessential hypertensionIncreased blood pressure
✦AI Summary

FBF1 (Fas binding factor 1) is a multifunctional protein primarily known as a transition fiber component essential for primary cilium organization and function. At the ciliary base, FBF1 localizes to distal appendages where it regulates cilia gating in coordination with TALPID3 and ANKRD26 1. FBF1 plays distinct roles in ciliary dynamics: it is specifically required for ciliary disassembly rather than assembly 2, and regulates intraflagellar transport and preciliary vesicle recruitment as part of a hierarchical protein assembly pathway 3. Beyond ciliary function, FBF1 has emerged as a critical senescence regulator. Under cellular stress, FBF1 undergoes SUMOylation at the ciliary base following downregulation of ciliary ARL GTPases, then translocates to promyelocytic leukemia nuclear bodies (PML-NBs) to initiate senescence responses 4. This translocation occurs via KIFC3-mediated transport along stress-induced nucleus-to-cilium microtubule arrays 5, and FBF1 ablation effectively reduces senescence burden in irradiation-treated mice 4. In adipose tissue, FBF1 constrains preadipocyte beiging through cilia-regulated PKA signaling and hedgehog pathway suppression 6. Additionally, FBF1 maintains cancer stem cell properties in breast cancer via PI3K/AKT/SOX2 signaling activation 7, suggesting therapeutic potential as a senotherapy and cancer treatment target.

Sources cited
1
FBF1 SUMOylation at ciliary base and PML-NB translocation initiates senescence; FBF1 ablation reduces senescence burden in mice
PMID: 37019904
2
KIFC3-mediated transport of FBF1 along polyglutamylated microtubule arrays is required for senescence induction
PMID: 39266565
3
TALPID3 and ANKRD26 coordinate FBF1 recruitment to transition fibers to regulate cilia gating function
PMID: 32366837
4
FBF1 specifically regulates ciliary disassembly and Aurora A kinase signaling at basal body
PMID: 39696441
5
FBF1 localizes to distal appendages and participates in hierarchical assembly pathway for cilia formation
PMID: 39882846
6
FBF1 constrains preadipocyte beiging through AKAP9-dependent PKA signaling and hedgehog pathway suppression
PMID: 34348145
7
FBF1 maintains breast cancer stem cell properties via PI3K/AKT/SOX2 signaling axis
PMID: 39988656
8
FBF1 inhibition promotes hepatocyte apoptosis and proliferation in LPS-induced acute liver injury
PMID: 39243556
Disease Associationsⓘ20
hypertensionOpen Targets
0.31Weak
cardiovascular diseaseOpen Targets
0.25Weak
essential hypertensionOpen Targets
0.23Weak
Increased blood pressureOpen Targets
0.21Weak
response to xenobiotic stimulusOpen Targets
0.15Weak
morbid obesityOpen Targets
0.11Weak
breast cancerOpen Targets
0.07Suggestive
alcohol drinkingOpen Targets
0.05Suggestive
hyperlipidemiaOpen Targets
0.05Suggestive
MODYOpen Targets
0.04Suggestive
diabetes mellitus, permanent neonatal 4Open Targets
0.04Suggestive
lipodystrophy, congenital generalized, type 5Open Targets
0.04Suggestive
permanent neonatal diabetes mellitus 1Open Targets
0.03Suggestive
Short stature, Dauber-Argente typeOpen Targets
0.03Suggestive
hyperinsulinemic hypoglycemia, familial, 8Open Targets
0.03Suggestive
hyperinsulinism due to INSR deficiencyOpen Targets
0.03Suggestive
obesity due to melanocortin 4 receptor deficiencyOpen Targets
0.03Suggestive
congenital analbuminemiaOpen Targets
0.03Suggestive
lipodystrophy, familial partial, type 8Open Targets
0.03Suggestive
mandibuloacral dysplasia with type A lipodystrophyOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
IFT20Protein interaction99%ODF2Protein interaction99%NINProtein interaction99%DXOProtein interaction84%XRN2Protein interaction84%IFT88Protein interaction82%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
57%
Heart
30%
Lung
30%
Liver
28%
Brain
24%
Gene Interaction Network
Click a node to explore
FBF1IFT20ODF2NINDXOXRN2IFT88
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q8TES7
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.13LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.97 [0.84–1.13]
RankingsWhere FBF1 stands among ~20K protein-coding genes
  • #10,003of 20,598
    Most Researched41
  • #11,718of 17,882
    Most Constrained (LOEUF)1.13
Genes detectedFBF1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
A stress-induced cilium-to-PML-NB route drives senescence initiation.
PMID: 37019904
Nat Commun · 2023
1.00
2
Transiently formed nucleus-to-cilium microtubule arrays mediate senescence initiation in a KIFC3-dependent manner.
PMID: 39266565
Nat Commun · 2024
0.90
3
TALPID3 and ANKRD26 selectively orchestrate FBF1 localization and cilia gating.
PMID: 32366837
Nat Commun · 2020
0.80
4
FBF1 maintains stem cell-like properties in breast cancer via PI3K/AKT/SOX2 axis.
PMID: 39988656
Stem Cell Res Ther · 2025
0.70
5
Distinct roles of centriole distal appendage proteins in ciliary assembly and disassembly.
PMID: 39696441
Cell Commun Signal · 2024
0.60