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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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PFKFB4
6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 4
Chromosome 3 · 3p21.31
NCBI Gene: 5210Ensembl: ENSG00000114268.12HGNC: HGNC:8875UniProt: B7Z5C3
76PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Kinase
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein binding6-phosphofructo-2-kinase activityfructose-2,6-bisphosphate 2-phosphatase activityfructose 2,6-bisphosphate metabolic processneoplasmbreast cancerhepatocellular carcinomaAbnormality of the skeletal system
✦AI Summary

PFKFB4 (6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 4) is a bifunctional metabolic enzyme that synthesizes and degrades fructose-2,6-bisphosphate, a potent allosteric activator of phosphofructokinase-1 and key regulator of glycolysis 1. Beyond its canonical metabolic role, PFKFB4 functions as a signaling hub that couples glucose metabolism to transcriptional reprogramming by phosphorylating the coactivator SRC-3 at serine 857, enhancing SRC-3-mediated transcriptional activity and promoting glucose flux toward anabolic pathways 23. PFKFB4 expression is upregulated across multiple cancer types including breast, cervical, endometrial, lung squamous cell carcinoma, pancreatic, and glioma, where it drives enhanced glycolysis and supports aggressive tumor phenotypes 24356. In glioma stem cells, PFKFB4 mRNA stabilization by lactylated PTBP1 sustains glycolytic reprogramming and stemness maintenance 6. PFKFB4 activity is regulated by phosphorylation; PIM2-mediated phosphorylation at threonine 140 promotes its function in endometriosis 7, while THOC3-YBX1 complexes control PFKFB4 mRNA export and stability 8. Clinical evidence demonstrates PFKFB4 elevation correlates with poor prognosis, metastasis, and advanced pathological stage across malignancies 243, positioning PFKFB4 as a promising therapeutic target for cancer treatment 5.

Sources cited
1
PFKFB4 (6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 4) is a bifunctional metabolic enzyme that synthesizes and degrades fructose-2,6-bisphosphate, a potent allosteric activator of phosphofructokinase-1 and key regulator of glycolysis .
PMID: 33671514
2
PFKFB4 expression is upregulated across multiple cancer types including breast, cervical, endometrial, lung squamous cell carcinoma, pancreatic, and glioma, where it drives enhanced glycolysis and supports aggressive tumor phenotypes , , , , .
PMID: 39570804
3
PFKFB4 activity is regulated by phosphorylation; PIM2-mediated phosphorylation at threonine 140 promotes its function in endometriosis , while THOC3-YBX1 complexes control PFKFB4 mRNA export and stability .
PMID: 36109523
4
PFKFB4 activity is regulated by phosphorylation; PIM2-mediated phosphorylation at threonine 140 promotes its function in endometriosis , while THOC3-YBX1 complexes control PFKFB4 mRNA export and stability .
PMID: 37500615
5
PFKFB4 expression is upregulated across multiple cancer types including breast, cervical, endometrial, lung squamous cell carcinoma, pancreatic, and glioma, where it drives enhanced glycolysis and supports aggressive tumor phenotypes , , , , .
PMID: 40832790
Disease Associationsⓘ20
neoplasmOpen Targets
0.11Weak
breast cancerOpen Targets
0.11Weak
hepatocellular carcinomaOpen Targets
0.11Weak
Abnormality of the skeletal systemOpen Targets
0.10Weak
melanomaOpen Targets
0.09Suggestive
health study participationOpen Targets
0.09Suggestive
triple-negative breast cancerOpen Targets
0.09Suggestive
non-small cell lung carcinomaOpen Targets
0.09Suggestive
lung adenocarcinomaOpen Targets
0.08Suggestive
glioblastoma multiformeOpen Targets
0.08Suggestive
gastric cancerOpen Targets
0.08Suggestive
small cell lung carcinomaOpen Targets
0.08Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.08Suggestive
endometriosisOpen Targets
0.07Suggestive
endometrial cancerOpen Targets
0.07Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.07Suggestive
gastroesophageal reflux diseaseOpen Targets
0.07Suggestive
cancerOpen Targets
0.07Suggestive
oral squamous cell carcinomaOpen Targets
0.07Suggestive
Female infertility due to fertilization defectOpen Targets
0.06Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PFKFB2Protein interaction98%PFKFB3Protein interaction98%PFKMProtein interaction98%HK2Protein interaction97%MPIProtein interaction97%TIGARProtein interaction97%
Tissue Expression6 tissues
Bone Marrow
100%
Lung
45%
Ovary
26%
Brain
17%
Liver
9%
Heart
7%
Gene Interaction Network
Click a node to explore
PFKFB4PFKFB2PFKFB3PFKMHK2MPITIGAR
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q16877
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.92LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.69 [0.53–0.92]
RankingsWhere PFKFB4 stands among ~20K protein-coding genes
  • #6,277of 20,598
    Most Researched76
  • #8,367of 17,882
    Most Constrained (LOEUF)0.92
Genes detectedPFKFB4
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
PTBP1 Lactylation Promotes Glioma Stem Cell Maintenance through PFKFB4-Driven Glycolysis.
PMID: 39570804
Cancer Res · 2025
1.00
2
Metabolic enzyme PFKFB4 activates transcriptional coactivator SRC-3 to drive breast cancer.
PMID: 29615789
Nature · 2018
0.90
3
Significance of PFKFB4 and gene risk signature in cervical cancer prognosis and progression.
PMID: 40466363
Biochem Biophys Res Commun · 2025
0.80
4
Phosphorylation of PFKFB4 by PIM2 promotes anaerobic glycolysis and cell proliferation in endometriosis.
PMID: 36109523
Cell Death Dis · 2022
0.70
5
THOC3 interacts with YBX1 to promote lung squamous cell carcinoma progression through PFKFB4 mRNA modification.
PMID: 37500615
Cell Death Dis · 2023
0.60