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50 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CCN2
cellular communication network factor 2
Chromosome 6 · 6q23.2
NCBI Gene: 1490Ensembl: ENSG00000118523.7HGNC: HGNC:2500UniProt: P29279
611PubMed Papers
22Diseases
1Drugs
3Pathogenic Variants
FUNCTIONAL ROLE
Hub Gene
RESEARCH IMPACT
Highly Studied
CLINICAL
Clinical TrialsOMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
extracellular matrixprotein bindingosteoblast differentiationpositive regulation of cell differentiationkyphomelic dysplasiaspondyloepimetaphyseal dysplasia, Li-Shao-Li typeidiopathic pulmonary fibrosispancreatic carcinoma
✦AI Summary

CCN2 (cellular communication network factor 2) is a secreted matricellular protein that functions as a critical hub in connective tissue development and pathological remodeling. Physiologically, CCN2 promotes osteoblast differentiation and osteogenesis through TGFBR/SMAD signaling 1, while also mediating cell adhesion and proliferation in fibroblasts, endothelial cells, and chondrocytes 2. The protein operates through multiple signaling cascades including ERK1/2, JNK, and integrin-dependent pathways 2. Pathologically, CCN2 drives fibrotic disease progression across multiple tissues. In knee arthrofibrosis, CCN2 expression is induced by TGF-β signaling in fibroblasts activated through macrophage interaction, promoting fibroblast pro-fibrotic functions and correlating with collagen deposition 3. In osteoarthritis, CCN2 facilitates IL-17 production and osteoclastogenesis via miR-655 inhibition 4. CCN2 also contributes to breast cancer osteolytic bone metastasis 5, APS vasculopathy through YAP1-mediated vascular smooth muscle cell proliferation 6, and atherosclerotic plaque vulnerability via endothelial-to-mesenchymal transition 7. Germline CCN2 variants cause spondyloepimetaphyseal dysplasia with low bone mass through impaired protein secretion and reduced osteogenic stimulation 1. CCN2 inhibition shows therapeutic potential in pulmonary fibrosis 8, making it a promising biomarker and therapeutic target across fibrotic and inflammatory diseases.

Sources cited
1
CCN2 has critical role in osteogenesis; monoallelic variants cause SEMD with low bone mass through impaired secretion
PMID: 39414788
2
CCN2 is a founding matricellular protein acting as signaling hub in skeletal development and pathological processes
PMID: 25294165
3
CCN2 mediates fibroblast-macrophage interaction in knee arthrofibrosis through TGF-β/SMAD pathway
PMID: 39994205
4
CCN2 facilitates IL-17 production and osteoclastogenesis in osteoarthritis synovial fibroblasts
PMID: 35876037
5
CCN2 overexpression contributes to osteolytic breast cancer bone metastasis
PMID: 12842083
6
CCN2 mediates APS vasculopathy through YAP1-dependent MVEC-smooth muscle cell signaling
PMID: 40878676
7
Endothelial CCN2 expression drives atherosclerotic plaque vulnerability via EndMT under disturbed flow conditions
PMID: 39818254
8
CCN2 inhibition is a therapeutic target for idiopathic pulmonary fibrosis
PMID: 40185752
Disease Associationsⓘ22
kyphomelic dysplasiaOpen Targets
0.43Moderate
spondyloepimetaphyseal dysplasia, Li-Shao-Li typeOpen Targets
0.33Weak
idiopathic pulmonary fibrosisOpen Targets
0.33Weak
pancreatic carcinomaOpen Targets
0.28Weak
cardiomyopathyOpen Targets
0.28Weak
enteritisOpen Targets
0.27Weak
vertebral column disorderOpen Targets
0.27Weak
pancreatic neoplasmOpen Targets
0.26Weak
Malignant Pancreatic NeoplasmOpen Targets
0.26Weak
COVID-19Open Targets
0.21Weak
secondary malignant neoplasmOpen Targets
0.19Weak
HyperhidrosisOpen Targets
0.19Weak
aortic stenosisOpen Targets
0.19Weak
Duchenne muscular dystrophyOpen Targets
0.18Weak
hemolytic anemiaOpen Targets
0.17Weak
nervous system benign neoplasmOpen Targets
0.14Weak
pulmonary embolismOpen Targets
0.12Weak
neoplasmOpen Targets
0.12Weak
hepatocellular carcinomaOpen Targets
0.11Weak
rheumatoid arthritisOpen Targets
0.11Weak
Kyphomelic dysplasiaUniProt
Spondyloepimetaphyseal dysplasia, Li-Shao-Li typeUniProt
Pathogenic Variants3
NM_001901.4(CCN2):c.65G>C (p.Arg22Pro)Pathogenic
Spondyloepimetaphyseal dysplasia, Li-Shao-Li type
☆☆☆☆2025→ Residue 22
NM_001901.4(CCN2):c.443G>A (p.Cys148Tyr)Pathogenic
Kyphomelic dysplasia
☆☆☆☆2025→ Residue 148
NM_001901.4(CCN2):c.779_786del (p.Pro260fs)Pathogenic
Kyphomelic dysplasia
☆☆☆☆2025→ Residue 260
View on ClinVar ↗
Drug Targets1
PAMREVLUMABPhase III
Connective tissue growth factor inhibitor
Malignant Pancreatic Neoplasm
Related Genes
EGFRProtein interaction100%TGFB1Protein interaction100%TGFBR1Protein interaction100%TGFBR2Protein interaction100%YAP1Protein interaction100%FN1Protein interaction100%
Tissue Expression6 tissues
Lung
100%
Heart
88%
Liver
38%
Ovary
32%
Brain
16%
Bone Marrow
4%
Gene Interaction Network
Click a node to explore
CCN2EGFRTGFB1TGFBR1TGFBR2YAP1FN1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt P29279
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.05LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.73 [0.52–1.05]
RankingsWhere CCN2 stands among ~20K protein-coding genes
  • #383of 20,598
    Most Researched611 · top 5%
  • #3,893of 5,498
    Most Pathogenic Variants3
  • #10,497of 17,882
    Most Constrained (LOEUF)1.05
Genes detectedCCN2
Sources retrieved50 papers
Response time—
📄 Sources
50â–¼
1
CCN2 mediates fibroblast-macrophage interaction in knee arthrofibrosis based on single-cell RNA-seq analysis.
PMID: 39994205
Bone Res · 2025
1.00
2
A multigenic program mediating breast cancer metastasis to bone.
PMID: 12842083
Cancer Cell · 2003
0.90
3
[Scleroderma].
PMID: 15954365
Nihon Rinsho · 2005
0.86
4
CCN2: A potential contributor to gingival overgrowth.
PMID: 39521130
J Oral Biosci · 2025
0.82
5
Microvascular Endothelial Cells License APS Vasculopathy Through YAP1- and CCN2-Mediated Signaling.
PMID: 40878676
Circulation · 2025
0.80