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25 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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PFKFB3
6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 3
Chromosome 10 · 10p15.1
NCBI Gene: 5209Ensembl: ENSG00000170525.21HGNC: HGNC:8874UniProt: A0A1W2PR17
211PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Kinase
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
fructose 2,6-bisphosphate metabolic processnucleoplasmprotein binding6-phosphofructo-2-kinase activityalcohol drinkingtype 1 diabetes mellitushypothyroidismrheumatoid arthritis
✦AI Summary

PFKFB3 is a key regulatory enzyme that catalyzes both the synthesis and degradation of fructose-2,6-bisphosphate, serving as a critical controller of glycolytic flux 1. The enzyme drives metabolic reprogramming by enhancing glycolysis while compromising mitochondrial respiration, particularly through hijacking glucose flux from the pentose phosphate pathway 2. PFKFB3 functions through multiple signaling pathways, including PI3K-Akt-mTOR activation, and its expression is post-transcriptionally regulated by CPEB4 binding to its 3'-UTR 34. The enzyme plays crucial roles in cellular processes beyond metabolism, regulating endothelial cell migration, filopodia formation, and vessel sprouting by compartmentalizing with F-actin in motile protrusions 5. PFKFB3 is significantly upregulated in various pathological conditions including sepsis, chr10 kidney disease, liver fibrosis, and pulmonary fibrosis 6147. Mechanistically, PFKFB3-driven glycolysis promotes inflammatory responses through histone lactylation-mediated NF-κB activation and facilitates pathological cell differentiation processes like endothelial-to-mesenchymal transition and myofibroblast activation 627. Therapeutic targeting of PFKFB3 with inhibitors shows promise in treating fibrotic diseases, sepsis, and metabolic disorders 81.

Sources cited
1
PFKFB3 is a critical engine that controls the rate of glycolysis and is upregulated in sepsis
PMID: 37199341
2
PFKFB3-driven glycolysis compromises mitochondrial respiration by hijacking glucose flux from pentose phosphate pathway
PMID: 36045132
3
PFKFB3 functions through PI3K-Akt-mTOR pathway activation
PMID: 32051533
4
PFKFB3 expression is post-transcriptionally regulated by CPEB4 binding to its 3'-UTR
PMID: 32169429
5
PFKFB3 regulates endothelial cell migration and vessel sprouting by compartmentalizing with F-actin
PMID: 23911327
6
PFKFB3 is upregulated in chronic kidney disease and promotes NF-κB activation through histone lactylation
PMID: 38789037
7
PFKFB3 is upregulated in pulmonary fibrosis and drives myofibroblast differentiation
PMID: 26284610
8
PFKFB3 inhibition shows therapeutic promise in treating metabolic disorders
PMID: 39294445
Disease Associationsⓘ20
alcohol drinkingOpen Targets
0.38Weak
type 1 diabetes mellitusOpen Targets
0.34Weak
hypothyroidismOpen Targets
0.34Weak
rheumatoid arthritisOpen Targets
0.33Weak
respiratory failureOpen Targets
0.31Weak
trauma complicationOpen Targets
0.30Weak
basal cell carcinomaOpen Targets
0.30Weak
neuroinflammatory disorderOpen Targets
0.28Weak
complicationOpen Targets
0.28Weak
medical procedureOpen Targets
0.28Weak
myxedemaOpen Targets
0.28Weak
autoimmune diseaseOpen Targets
0.24Weak
type 2 diabetes mellitusOpen Targets
0.21Weak
celiac diseaseOpen Targets
0.21Weak
asthmaOpen Targets
0.20Weak
cervical carcinomaOpen Targets
0.16Weak
cancerOpen Targets
0.11Weak
neoplasmOpen Targets
0.11Weak
breast cancerOpen Targets
0.11Weak
hepatocellular carcinomaOpen Targets
0.11Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PFKFB2Protein interaction98%PFKFB4Protein interaction98%PFKMProtein interaction98%HK1Protein interaction97%HK2Protein interaction97%MPIProtein interaction97%
Tissue Expression6 tissues
Bone Marrow
100%
Lung
62%
Brain
33%
Ovary
17%
Liver
15%
Heart
8%
Gene Interaction Network
Click a node to explore
PFKFB3PFKFB2PFKFB4PFKMHK1HK2MPI
PROTEIN STRUCTURE
Preparing viewer…
PDB6HVI · 1.96 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.62LoF Tolerant
pLIⓘ
0.01Tolerant
Observed/Expected LoF0.46 [0.35–0.62]
RankingsWhere PFKFB3 stands among ~20K protein-coding genes
  • #1,979of 20,598
    Most Researched211 · top 10%
  • #4,321of 17,882
    Most Constrained (LOEUF)0.62 · top quartile
Genes detectedPFKFB3
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
The glycolytic enzyme PFKFB3 drives kidney fibrosis through promoting histone lactylation-mediated NF-κB family activation.
PMID: 38789037
Kidney Int · 2024
1.00
2
Role of PFKFB3-driven glycolysis in sepsis.
PMID: 37199341
Ann Med · 2023
0.90
3
Role of PFKFB3-driven glycolysis in vessel sprouting.
PMID: 23911327
Cell · 2013
0.80
4
Cyclosporine modulates neutrophil functions via the SIRT6-HIF-1α-glycolysis axis to alleviate severe ulcerative colitis.
PMID: 33634990
Clin Transl Med · 2021
0.72
5
Suppression of PFKFB3-driven glycolysis restrains endothelial-to-mesenchymal transition and fibrotic response.
PMID: 36045132
Signal Transduct Target Ther · 2022
0.70