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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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CHPF2
chondroitin polymerizing factor 2
Chromosome 7 · 7q36.1
NCBI Gene: 54480Ensembl: ENSG00000033100.17HGNC: HGNC:29270UniProt: A0A8I5KRN5
35PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
acetylgalactosaminyltransferase activityprotein-macromolecule adaptor activityN-acetylgalactosaminyl-proteoglycan 3-beta-glucuronosyltransferase activitychondroitin sulfate proteoglycan biosynthetic processneurodegenerative diseaseAbnormality of the skeletal systemsmoking initiationmultiple myeloma
✦AI Summary

CHPF2 (chondroitin polymerizing factor 2) is a glucuronosyltransferase that transfers glucuronic acid from UDP-GlcUA to N-acetylgalactosamine residues on elongating chondroitin polymer chains 1. However, recent structural analysis reveals CHPF2 primarily plays a stabilizing role within heterodimeric complexes (CHSY1-CHPF2 and CHSY3-CHPF2) rather than serving as the catalytic enzyme; only CHSY1 and CHSY3 demonstrate bifunctional glycosyltransferase activities 1. CHPF2 is enriched in astrocytes and regulates chondroitin sulfate glycosaminoglycan biosynthesis, with astrocyte-derived chondroitin sulfate inhibiting neuronal dendritic development and plasticity 23. Beyond its canonical enzymatic role, CHPF2 exhibits a moonlighting function independent of enzymatic activity: upon TNF stimulation, MEK phosphorylates CHPF2 at T588, enabling interaction with TAK1 and IKKα to amplify NF-κB signaling and promote colorectal cancer cell proliferation and metastasis 4. CHPF2 is dysregulated in multiple cancers, serving as a biomarker in hepatocellular carcinoma and bladder cancer with prognostic significance 56. Additionally, variants in CHPF2 accumulate in thrombotic storm patients, suggesting roles in regulating endothelial coagulation control 7. Exosomal lncRNA DUXAP8 regulates CHPF2 expression in intracranial aneurysm pathogenesis 8.

Sources cited
1
CHPF2 plays a stabilizing role within heterodimeric enzyme complexes; only CHSY partners possess catalytic activity for chondroitin sulfate chain polymerization
PMID: 41298522
2
CHPF2 is enriched in astrocytes and regulates chondroitin sulfate biosynthesis, which inhibits neuronal dendritic development
PMID: 40192069
3
Chpf2 translation in astrocytes regulates dendritic arborization in developing neurons through chondroitin sulfate glycosaminoglycan synthesis
PMID: 39211148
4
CHPF2 phosphorylation at T588 by MEK promotes colorectal cancer cell proliferation and metastasis through NF-κB signaling activation, independent of enzymatic activity
PMID: 38253217
5
CHPF2 is a novel biomarker in hepatocellular carcinoma; knockdown of CHPF2 inhibits HCC cell proliferation and migration
PMID: 40107635
6
CHPF2 is a core gene in ferroptosis-glycolysis related gene sets and serves as a prognostic biomarker in bladder cancer
PMID: 40401561
7
Conserved rare variants in CHPF2 accumulate in thrombotic storm patients, suggesting roles in endothelial coagulation control through chondroitin sulfate metabolism
PMID: 29178990
8
Exosomal lncRNA DUXAP8 regulates CHPF2 expression in intracranial aneurysm pathogenesis
PMID: 38341004
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.29Weak
Abnormality of the skeletal systemOpen Targets
0.14Weak
smoking initiationOpen Targets
0.12Weak
multiple myelomaOpen Targets
0.08Suggestive
lymphoid neoplasmOpen Targets
0.06Suggestive
coronary artery diseaseOpen Targets
0.05Suggestive
cholelithiasisOpen Targets
0.03Suggestive
coronary atherosclerosisOpen Targets
0.03Suggestive
malignant pleural mesotheliomaOpen Targets
0.02Suggestive
hepatocellular carcinomaOpen Targets
0.02Suggestive
glioblastoma multiformeOpen Targets
0.02Suggestive
colorectal carcinomaOpen Targets
0.01Suggestive
brain aneurysmOpen Targets
0.01Suggestive
colorectal cancerOpen Targets
0.01Suggestive
melanomaOpen Targets
0.01Suggestive
cancerOpen Targets
0.00Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.00Suggestive
breast cancerOpen Targets
0.00Suggestive
papillary thyroid carcinomaOpen Targets
0.00Suggestive
thyroid cancerOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CHPFProtein interaction93%CSGALNACT1Protein interaction93%CHSY3Protein interaction90%CSGALNACT2Protein interaction71%CHSY1Protein interaction56%SLC35B2Shared pathway50%
Tissue Expression6 tissues
Lung
100%
Bone Marrow
92%
Heart
74%
Liver
69%
Ovary
64%
Brain
33%
Gene Interaction Network
Click a node to explore
CHPF2CHPFCSGALNACT1CHSY3CSGALNACT2CHSY1SLC35B2
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q9P2E5
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.14LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.91 [0.74–1.14]
RankingsWhere CHPF2 stands among ~20K protein-coding genes
  • #10,935of 20,598
    Most Researched35
  • #11,769of 17,882
    Most Constrained (LOEUF)1.14
Genes detectedCHPF2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
CHPF2 as a novel biomarker and ponicidin as a potential therapeutic agent in hepatocellular carcinoma.
PMID: 40107635
Pharmacol Res · 2025
1.00
2
Structural basis for human chondroitin sulfate chain polymerization.
PMID: 41298522
Nat Commun · 2025
0.90
3
Astrocyte Extracellular Matrix Modulates Neuronal Dendritic Development.
PMID: 40192069
Glia · 2025
0.80
4
Discovery and Validation of a New Biomarker Integrating Ferroptosis and Glycolysis-Related Genes in Bladder Cancer.
PMID: 40401561
IUBMB Life · 2025
0.70
5
MEK-mediated CHPF2 phosphorylation promotes colorectal cancer cell proliferation and metastasis by activating NF-κB signaling.
PMID: 38253217
Cancer Lett · 2024
0.60