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25 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ATP1B1
ATPase Na+/K+ transporting subunit beta 1
Chromosome 1 · 1q24.2
NCBI Gene: 481Ensembl: ENSG00000143153.15HGNC: HGNC:804UniProt: A3KLL5
166PubMed Papers
20Diseases
6Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedTransporter
RESEARCH IMPACT
Trending
CLINICAL
FDA Approved Target
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
membrane repolarizationmembraneATPase activator activityplasma membranecardiovascular diseaseatrial fibrillationcongestive heart failureheart failure
✦AI Summary

ATP1B1 encodes the β1 subunit of the Na+/K+-ATPase pump, a critical regulator of cellular ion homeostasis and membrane potential across diverse tissues. The protein functions as an obligate component of the heterodiomeric Na+/K+-ATPase complex with the α-subunit ATP1A1, maintaining intracellular sodium and potassium gradients essential for cell survival 1. Beyond its canonical role in ion transport, ATP1B1 participates in establishing epithelial cell polarity and cell adhesion, with localized expression in cochlear hair cells, supporting cells, and spiral ganglion neurons where it supports auditory nerve signaling 2. Emerging evidence reveals ATP1B1's involvement in antiviral innate immunity; upon viral infection, ATP1B1 is upregulated and interacts with TRAF3 and TRAF6 to potentiate their ubiquitination, enhancing interferon and inflammatory cytokine production 3. Additionally, ATP1B1 serves as a fusion partner in oncogenic rearrangements, including ATP1B1-NRG1 and ATP1B1-PRKACA fusions identified in pancreatic and pancreatobiliary cancers, where these fusions constitute therapeutic targets 4, 5, 6. The protein also interacts with human cytomegalovirus UL136 protein, suggesting roles in viral pathogenesis and cellular ion homeostasis during infection 7. Disease associations include cardiovascular disorders, cataracts, and hematologic malignancies, reflecting ATP1B1's tissue-wide importance in maintaining cellular homeostasis.

Sources cited
1
ATP1B1 is the β-subunit of the Na+/K+-ATPase pump that interacts with ATP1A1 to maintain cellular homeostasis and membrane potential
PMID: 39024560
2
ATP1B1 gene transcripts are expressed in cochlear hair cells, supporting cells, and spiral ganglion neurons, essential for cochlear function
PMID: 35431803
3
ATP1B1 is upregulated after viral infection and potentiates ubiquitination of TRAF3 and TRAF6 to enhance interferon and inflammatory cytokine production
PMID: 34011520
4
ATP1B1 is identified as a fusion partner with NRG1 in lung cancer and serves as a therapeutic target for ErbB-targeted treatments
PMID: 32916265
5
ATP1B1-NRG1 fusions occur in pancreatic cancer and are responsive to HER2/HER3 bispecific antibody therapy
PMID: 35135829
6
ATP1B1 serves as a fusion partner with PRKACA and PRKACB in intraductal oncocytic papillary neoplasms of pancreatic and biliary origin
PMID: 37871652
7
ATP1B1 interacts with human cytomegalovirus UL136 protein and may have roles in cell osmotic pressure and intracellular ion homeostasis during HCMV infection
PMID: 22030864
Disease Associationsⓘ20
cardiovascular diseaseOpen Targets
0.64Moderate
atrial fibrillationOpen Targets
0.61Moderate
congestive heart failureOpen Targets
0.60Moderate
heart failureOpen Targets
0.58Moderate
thrombophiliaOpen Targets
0.51Moderate
ArrhythmiaOpen Targets
0.50Moderate
coronary artery diseaseOpen Targets
0.49Moderate
Fuchs' endothelial dystrophyOpen Targets
0.44Moderate
coronary atherosclerosisOpen Targets
0.41Moderate
corneal diseaseOpen Targets
0.41Moderate
corneal dystrophyOpen Targets
0.40Moderate
blood coagulation diseaseOpen Targets
0.39Weak
ThromboembolismOpen Targets
0.38Weak
Fuchs endothelial corneal dystrophyOpen Targets
0.36Weak
cataractOpen Targets
0.36Weak
angina pectorisOpen Targets
0.36Weak
venous thromboembolismOpen Targets
0.33Weak
iron metabolism diseaseOpen Targets
0.31Weak
keratoconusOpen Targets
0.30Weak
Age-related cataractOpen Targets
0.29Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Drug Targets6
ACETYLDIGITOXINApproved
Sodium/potassium-transporting ATPase inhibitor
cardiovascular disease
DESLANOSIDEApproved
Sodium/potassium-transporting ATPase inhibitor
cardiovascular disease
DIGITOXINApproved
Sodium/potassium-transporting ATPase inhibitor
cardiovascular disease
DIGOXINApproved
Sodium/potassium-transporting ATPase inhibitor
heart failure
ISTAROXIMEPhase II
Sarcoplasmic/endoplasmic reticulum calcium ATPase 2 activator
heart disease
LANATOSIDE CApproved
Sodium/potassium-transporting ATPase inhibitor
cardiovascular disease
Related Genes
MAPK3Protein interaction99%ABCA2Protein interaction96%PRKACAProtein interaction93%PRKACBProtein interaction93%SLC9A1Protein interaction92%NKAIN1Protein interaction92%
Tissue Expression6 tissues
Brain
100%
Heart
59%
Liver
23%
Ovary
13%
Lung
9%
Bone Marrow
3%
Gene Interaction Network
Click a node to explore
ATP1B1MAPK3ABCA2PRKACAPRKACBSLC9A1NKAIN1
PROTEIN STRUCTURE
Preparing viewer…
PDB8ZYJ · 2.37 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.20Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.08 [0.03–0.20]
RankingsWhere ATP1B1 stands among ~20K protein-coding genes
  • #2,682of 20,598
    Most Researched166 · top quartile
  • #291of 1,025
    FDA-Approved Drug Targets5
  • #420of 17,882
    Most Constrained (LOEUF)0.20 · top 5%
Genes detectedATP1B1
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
NRG1 fusion-driven tumors: biology, detection, and the therapeutic role of afatinib and other ErbB-targeting agents.
PMID: 32916265
Ann Oncol · 2020
1.00
2
Targeting the Sodium-Potassium Pump as a Therapeutic Strategy in Acute Myeloid Leukemia.
PMID: 39024560
Cancer Res · 2024
0.90
3
Zenocutuzumab, a HER2xHER3 Bispecific Antibody, Is Effective Therapy for Tumors Driven by NRG1 Gene Rearrangements.
PMID: 35135829
Cancer Discov · 2022
0.80
4
Increased Protein Kinase A Activity Induces Fibrolamellar Hepatocellular Carcinoma Features Independent of DNAJB1.
PMID: 38888469
Cancer Res · 2024
0.76
5
Interaction between human cytomegalovirus UL136 protein and ATP1B1 protein.
PMID: 22030864
Braz J Med Biol Res · 2011
0.70