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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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NAB2
NGFI-A binding protein 2
Chromosome 12 · 12q13.3
NCBI Gene: 4665Ensembl: ENSG00000166886.14HGNC: HGNC:7627UniProt: Q15742
77PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingidentical protein bindingnegative regulation of transcription by RNA polymerase IIIregulation of DNA-templated transcriptionsolitary fibrous tumormeningiomahemangiopericytomameninges hemangiopericytoma
✦AI Summary

NAB2 (NGFI-A binding protein 2) functions as a transcriptional corepressor that regulates zinc finger transcription factors and controls gene expression through multiple mechanisms. In normal cellular contexts, NAB2 acts as a negative regulator of transcription, with specific isoforms exhibiting differential repression capacity. Clinically, NAB2 is most significant in its pathogenic role through fusion with STAT6. The NAB2-STAT6 fusion is the defining molecular alteration of solitary fibrous tumors (SFTs), a soft tissue sarcoma 12345. This fusion event leads to nuclear relocalization of STAT6 protein, detectable by immunohistochemistry, with exon 4-STAT6 exon 3 fusions correlating with classic SFT morphology and potentially less aggressive behavior 5. NAB2-STAT6 fusions have been identified in meningeal and non-meningeal SFTs as well as hemangiopericytomas, unifying these previously distinct entities into a single disease continuum 45. Importantly, NAB2-STAT6 fusion status is used for risk stratification in SFTs to predict metastatic potential 1. Beyond neoplasia, NAB2 regulates intramuscular adipogenesis by driving CFD expression in fibro-adipogenic progenitors, with elevated CFD-NAB2 signaling observed in human metabolic syndrome and Duchenne muscular dystrophy, correlating with pathological fat accumulation 6.

Sources cited
1
NAB2-STAT6 gene fusion characterizes solitary fibrous tumors; fusion status correlates with metastatic risk stratification
PMID: 28731041
2
NAB2-STAT6 fusion is a novel genetic alteration of diagnostic relevance in solitary fibrous tumor classification
PMID: 32796172
3
NAB2-STAT6 gene fusion is the defining molecular marker of solitary fibrous tumor with emerging clinical significance
PMID: 33879215
4
NAB2-STAT6 fusion is the definitive molecular alteration in both solitary fibrous tumor and hemangiopericytoma, leading to STAT6 nuclear relocation
PMID: 35169094
5
NAB2-STAT6 fusion variants (exon 4-exon 3, exon 6-exon 17, exon 6-exon 18) correlate with tumor morphology and behavior in meningeal tumors
PMID: 26883114
6
NAB2 drives CFD expression in fibro-adipogenic progenitors promoting adipogenesis; elevated in metabolic syndrome and DMD patients
PMID: 37439037
Disease Associationsⓘ20
solitary fibrous tumorOpen Targets
0.38Weak
meningiomaOpen Targets
0.37Weak
hemangiopericytomaOpen Targets
0.37Weak
meninges hemangiopericytomaOpen Targets
0.37Weak
Dedifferentiated Solitary Fibrous TumorOpen Targets
0.37Weak
pleural solitary fibrous tumorOpen Targets
0.37Weak
T-Cell Prolymphocytic LeukemiaOpen Targets
0.37Weak
thyroid neoplasmOpen Targets
0.37Weak
Transitional MeningiomaOpen Targets
0.37Weak
head and neck squamous cell carcinomaOpen Targets
0.29Weak
oral squamous cell carcinomaOpen Targets
0.28Weak
prostate carcinomaOpen Targets
0.28Weak
gliomaOpen Targets
0.28Weak
acute myeloid leukemiaOpen Targets
0.28Weak
hepatocellular carcinomaOpen Targets
0.28Weak
angiosarcomaOpen Targets
0.28Weak
Fibrous MeningiomaOpen Targets
0.28Weak
carcinoma of liver and intrahepatic biliary tractOpen Targets
0.28Weak
Endometrial Endometrioid AdenocarcinomaOpen Targets
0.28Weak
lung adenocarcinomaOpen Targets
0.28Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
EGR4Protein interaction99%EGR1Protein interaction99%EGR2Protein interaction99%EGR3Protein interaction99%STAT6Protein interaction93%ZC3H14Protein interaction85%
Tissue Expression6 tissues
Ovary
100%
Lung
43%
Liver
40%
Heart
31%
Bone Marrow
27%
Brain
13%
Gene Interaction Network
Click a node to explore
NAB2EGR4EGR1EGR2EGR3STAT6ZC3H14
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q15742
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.40Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.23 [0.14–0.40]
RankingsWhere NAB2 stands among ~20K protein-coding genes
  • #6,187of 20,598
    Most Researched77
  • #2,030of 17,882
    Most Constrained (LOEUF)0.40 · top quartile
Genes detectedNAB2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Risk assessment in solitary fibrous tumors: validation and refinement of a risk stratification model.
PMID: 28731041
Mod Pathol · 2017
1.00
2
The 2020 WHO Classification: What's New in Soft Tissue Tumor Pathology?
PMID: 32796172
Am J Surg Pathol · 2021
0.90
3
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
PMID: 33879215
Diagn Pathol · 2021
0.80
4
A single-cell atlas of bovine skeletal muscle reveals mechanisms regulating intramuscular adipogenesis and fibrogenesis.
PMID: 37439037
J Cachexia Sarcopenia Muscle · 2023
0.70
5
[Solitary fibrous tumor/hemangiopericytoma].
PMID: 35169094
No Shinkei Geka · 2022
0.60