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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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GFPT2
glutamine--fructose-6-phosphate transaminase 2
Chromosome 5 · 5q35.3
NCBI Gene: 9945Ensembl: ENSG00000131459.14HGNC: HGNC:4242UniProt: A0A0S2Z4X9
60PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
UDP-N-acetylglucosamine biosynthetic processprotein bindingL-glutamine:D-fructose-6-phosphate transaminase (isomerizing) activityfructose 6-phosphate metabolic processprimary thrombocytopeniacholelithiasispneumonitisneoplasm
✦AI Summary

GFPT2 (glutamine-fructose-6-phosphate transaminase 2) is the rate-limiting enzyme of the hexosamine biosynthetic pathway (HBP), catalyzing conversion of fructose-6-phosphate and glutamine to glucosamine-6-phosphate and thereby controlling glucose flux into this pathway 1. Via HBP regulation, GFPT2 likely controls precursor availability for N- and O-linked protein glycosylation 1. Unlike its paralog GFPT1, GFPT2 exhibits substantially reduced feedback inhibition by UDP-N-acetylglucosamine, enabling sustained pathway activation when paired with AMDHD2 1. GFPT2 has emerged as a critical metabolic vulnerability in multiple malignancies. In KRAS/LKB1 co-mutant lung cancer, GFPT2 inhibition selectively reduces tumor growth in vitro and in vivo 2. In pancreatic cancer, GFPT2-mediated O-GlcNAcylation of YBX1 promotes IL-18 transcription, facilitating M2 macrophage polarization and immunosuppression 3. GFPT2 overexpression in tumor cells impairs macrophage mitochondrial fission needed for antibody-mediated phagocytosis, representing a targetable immune evasion mechanism 4. In mesothelioma, GFPT2 activates NF-κB signaling through p65 O-GlcNAcylation, promoting malignant progression with 92.6% diagnostic sensitivity 5. Genetically, GFPT2 polymorphisms associate with type 2 diabetes and diabetic nephropathy, with certain variants increasing mRNA levels and hexosamine flux 6.

Sources cited
1
GFPT2 is the rate-limiting enzyme of HBP; catalyzes glucosamine-6-phosphate formation; exhibits reduced feedback inhibition compared to GFPT1
PMID: 35229715
2
GFPT2 inhibition selectively reduces KRAS/LKB1 co-mutant tumor growth in culture, xenografts, and genetically modified mice
PMID: 33257855
3
GFPT2-mediated O-GlcNAcylation of YBX1 promotes IL-18 transcription and macrophage M2 polarization in pancreatic cancer
PMID: 38575607
4
GFPT2 overexpression in tumor cells impairs macrophage mitochondrial fission and antibody-mediated phagocytosis
PMID: 35484420
5
GFPT2 shows 92.6% diagnostic sensitivity for mesothelioma; drives malignant progression via NF-κB-p65 O-GlcNAcylation
PMID: 39317702
6
GFPT2 polymorphisms associate with type 2 diabetes and diabetic nephropathy; certain variants increase mRNA levels
PMID: 14764791
Disease Associationsⓘ20
primary thrombocytopeniaOpen Targets
0.32Weak
cholelithiasisOpen Targets
0.27Weak
pneumonitisOpen Targets
0.19Weak
neoplasmOpen Targets
0.10Suggestive
glomerulonephritisOpen Targets
0.08Suggestive
non-small cell lung carcinomaOpen Targets
0.08Suggestive
gastric cancerOpen Targets
0.08Suggestive
breast cancerOpen Targets
0.06Suggestive
Familial exudative vitreoretinopathyOpen Targets
0.04Suggestive
ovarian cancerOpen Targets
0.04Suggestive
glioblastoma multiformeOpen Targets
0.04Suggestive
otodental syndromeOpen Targets
0.04Suggestive
amelogenesis imperfectaOpen Targets
0.04Suggestive
retinitis pigmentosa and erythrocytic microcytosisOpen Targets
0.04Suggestive
OligodontiaOpen Targets
0.04Suggestive
tooth agenesisOpen Targets
0.04Suggestive
Parkinson diseaseOpen Targets
0.04Suggestive
exfoliation syndromeOpen Targets
0.03Suggestive
Genetic malformation syndrome with short statureOpen Targets
0.03Suggestive
Familial ocular anterior segment mesenchymal dysgenesisOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
HK1Protein interaction99%HK2Protein interaction99%PGM3Protein interaction99%UAP1Protein interaction99%GPIProtein interaction99%GNPDA1Protein interaction97%
Tissue Expression6 tissues
Ovary
100%
Lung
45%
Heart
18%
Brain
13%
Liver
4%
Bone Marrow
1%
Gene Interaction Network
Click a node to explore
GFPT2HK1HK2PGM3UAP1GPIGNPDA1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt O94808
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.13LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.86 [0.67–1.13]
RankingsWhere GFPT2 stands among ~20K protein-coding genes
  • #7,663of 20,598
    Most Researched60
  • #11,635of 17,882
    Most Constrained (LOEUF)1.13
Genes detectedGFPT2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The hexosamine biosynthesis pathway is a targetable liability in KRAS/LKB1 mutant lung cancer.
PMID: 33257855
Nat Metab · 2020
1.00
2
The GFPT2-O-GlcNAcylation-YBX1 axis promotes IL-18 secretion to regulate the tumor immune microenvironment in pancreatic cancer.
PMID: 38575607
Cell Death Dis · 2024
0.90
3
Macrophage mitochondrial fission improves cancer cell phagocytosis induced by therapeutic antibodies and is impaired by glutamine competition.
PMID: 35484420
Nat Cancer · 2022
0.80
4
GFPT2: A novel biomarker in mesothelioma for diagnosis and prognosis and its molecular mechanism in malignant progression.
PMID: 39317702
Br J Cancer · 2024
0.70
5
Nutlin-3a induces KRAS mutant/p53 wild type lung cancer specific methuosis-like cell death that is dependent on GFPT2.
PMID: 38093368
J Exp Clin Cancer Res · 2023
0.60