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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ATF1
activating transcription factor 1
Chromosome 12 · 12q13.12
NCBI Gene: 466Ensembl: ENSG00000123268.10HGNC: HGNC:783UniProt: P18846
131PubMed Papers
21Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
RNA polymerase II transcription regulatory region sequence-specific DNA bindingDNA-binding transcription activator activity, RNA polymerase II-specificprotein bindingidentical protein bindingcolorectal cancerhypertensionmelanomaclear cell sarcoma
✦AI Summary

ATF1 is a cAMP-responsive transcription factor that binds cAMP response elements (CRE) in gene promoters and mediates PKA-induced transcriptional activation 1. It functions as part of transcription factor complexes with ATF4 and CREB1, participating in cAMP/PKA signal transduction pathways 2. In normal physiology, ATF1 regulates anti-inflammatory microglial responses in neuroinflammatory conditions, where it serves as a key regulator of anti-inflammatory disease-associated microglia phenotypes 2. ATF1 also promotes colorectal cancer progression through direct transcriptional regulation of PRRC2A, which activates oncogenic WNT and YAP signaling 3. Clinically, ATF1 is predominantly recognized through its involvement in sarcomas and mesotheliomas. ATF1 undergoes recurrent fusion with EWSR1/FUS genes in soft-tissue neoplasms, including clear cell sarcomas, angiomatoid fibrous histiocytomas, and mesothelial tumors, generating chimeric transcription factors that drive malignant transformation 45678. EWSR1-ATF1 fusion tumors occur predominantly in young patients and show aggressive biological behavior with propensity for local recurrence and poor prognosis 46. ATF1 rearrangements represent important diagnostic and prognostic markers in mesothelial lesions, distinguishing them from conventional mesotheliomas 7. Recognition of ATF1-fusion status is essential for accurate diagnosis and risk stratification in young patients with mesenchymal malignancies.

Sources cited
1
ATF1 is a regulator of anti-inflammatory disease-associated microglia and participates in transcription factor complexes in neuroinflammatory conditions
PMID: 29784049
2
ATF1 is phosphorylated by PKA and PKD downstream of stress and obesity signals in pancreatic cancer pathogenesis
PMID: 41484057
3
EWSR1-ATF1 fusion occurs in intracranial mesenchymal tumors, predominantly in young patients, with epithelioid morphology and poor prognosis
PMID: 33141488
4
EWSR1/FUS-ATF1 fusions are specifically associated with peritoneal mesothelioma in young patients
PMID: 36729766
5
EWSR1/FUS::ATF1 fusions display angiomatoid fibrous histiocytoma-like patterns and represent poor prognosis factors in mesotheliomas
PMID: 39059063
6
ATF1 rearrangement should be considered in young mesothelioma patients without significant asbestos exposure as an uncommon genetic alteration
PMID: 38190277
7
EWSR1::ATF1 fusion generates a chimeric transcription factor that initiates cancer formation through dysregulated gene expression in soft-tissue neoplasms
PMID: 39769457
8
ATF1 directly regulates PRRC2A transcription in colorectal cancer, promoting tumor progression via WNT and YAP signaling
PMID: 39582289
Disease Associationsⓘ21
colorectal cancerOpen Targets
0.44Moderate
hypertensionOpen Targets
0.41Moderate
melanomaOpen Targets
0.38Weak
clear cell sarcomaOpen Targets
0.37Weak
cutaneous melanomaOpen Targets
0.37Weak
synovial sarcomaOpen Targets
0.37Weak
liposarcomaOpen Targets
0.37Weak
cutaneous fibrous histiocytomaOpen Targets
0.37Weak
lymphoid neoplasmOpen Targets
0.37Weak
malignant peripheral nerve sheath tumorOpen Targets
0.37Weak
malignant soft tissue neoplasmOpen Targets
0.37Weak
mixed liposarcomaOpen Targets
0.37Weak
Ocular MelanomaOpen Targets
0.37Weak
Ovarian Endometrioid Adenocarcinoma with Squamous DifferentiationOpen Targets
0.37Weak
undifferentiated pleomorphic sarcomaOpen Targets
0.37Weak
Varicose veinsOpen Targets
0.33Weak
Abnormality of the skeletal systemOpen Targets
0.32Weak
hepatocellular carcinomaOpen Targets
0.30Weak
lung adenocarcinomaOpen Targets
0.30Weak
non-small cell lung carcinomaOpen Targets
0.28Weak
Angiomatoid fibrous histiocytomaUniProt
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CREB3Protein interaction96%EWSR1Protein interaction95%CREB3L1Protein interaction95%CREB3L2Protein interaction94%ATF4Protein interaction94%ATF6BProtein interaction93%
Tissue Expression6 tissues
Heart
100%
Ovary
77%
Lung
68%
Liver
63%
Bone Marrow
56%
Brain
42%
Gene Interaction Network
Click a node to explore
ATF1CREB3EWSR1CREB3L1CREB3L2ATF4ATF6B
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt P18846
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.68LoF Tolerant
pLIⓘ
0.10Tolerant
Observed/Expected LoF0.44 [0.30–0.68]
RankingsWhere ATF1 stands among ~20K protein-coding genes
  • #3,546of 20,598
    Most Researched131 · top quartile
  • #5,098of 17,882
    Most Constrained (LOEUF)0.68
Genes detectedATF1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Identification and therapeutic modulation of a pro-inflammatory subset of disease-associated-microglia in Alzheimer's disease.
PMID: 29784049
Mol Neurodegener · 2018
1.00
2
Pancreatic cancer: molecular pathogenesis and emerging therapeutic strategies.
PMID: 41484057
Signal Transduct Target Ther · 2026
0.90
3
Cholesterol metabolism regulated by CAMKK2-CREB signaling promotes castration-resistant prostate cancer.
PMID: 40483692
Cell Rep · 2025
0.80
4
Intracranial mesenchymal tumor with FET-CREB fusion-A unifying diagnosis for the spectrum of intracranial myxoid mesenchymal tumors and angiomatoid fibrous histiocytoma-like neoplasms.
PMID: 33141488
Brain Pathol · 2021
0.70
5
Peritoneal Mesothelioma-An Update.
PMID: 36729766
Adv Anat Pathol · 2023
0.60