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9 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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PABIR1
PP2A Aalpha (PPP2R1A) and B55A (PPP2R2A) interacting phosphatase regulator 1
Chromosome 9 · 9q21.11
NCBI Gene: 116224Ensembl: ENSG00000187866.10HGNC: HGNC:23490UniProt: B3KX07
34PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein phosphatase inhibitor activityprotein serine/threonine phosphatase inhibitor activityprotein bindingprotein phosphatase 2A bindinggoutneuroendocrine neoplasmAbnormal pupillary functiondiabetes mellitus
✦AI Summary

PABIR1 (FAM122A) is an intrinsically disordered protein that functions as an endogenous inhibitor of protein phosphatase 2A (PP2A), specifically the PP2A:B55α complex 1. PABIR1 inhibits PP2A through a competitive mechanism: it contains conserved helical motifs ([RK]-V-x-x-[VI]-R) that bind to B55α and block substrate docking sites while spatially constraining access to the catalytic subunit 23. By inhibiting PP2A-mediated dephosphorylation of WEE1, PABIR1 promotes WEE1 ubiquitin-dependent degradation and facilitates G2/M checkpoint release during mitotic entry 4. PABIR1 also potentiates PP2A catalytic subunit degradation through increased poly-ubiquitination 1. Functionally, PABIR1 is essential for cell cycle progression through G1/S and intra-S checkpoints, and its deletion impairs checkpoint control in response to replication stress 3. Clinically, PABIR1 expression correlates with cancer cell proliferation and survival: it is upregulated in hepatocellular carcinoma, and its deletion enhances chemotherapy sensitivity 5. PABIR1 expression levels serve as a biomarker for predicting CHK1 inhibitor sensitivity in tumor cells 4.

Sources cited
1
PABIR1 (FAM122A) directly interacts with PP2A subunits and inhibits PP2A activity, potentiates PP2A catalytic subunit degradation
PMID: 27588481
2
Cryo-EM structures reveal how PABIR1 as an intrinsically disordered protein binds PP2A:B55 to inhibit its activity
PMID: 38123684
3
PABIR1 contains helical motifs that competitively block substrate binding and dephosphorylation by B55α/PP2A; essential for cell cycle checkpoint control
PMID: 38982062
4
PABIR1 inhibits PP2A-mediated WEE1 dephosphorylation, promoting WEE1 degradation and G2/M checkpoint release; PABIR1 expression predicts CHK1 inhibitor sensitivity
PMID: 33108758
5
PABIR1 is upregulated in hepatocellular carcinoma; its deletion inhibits tumor cell growth and enhances chemotherapy sensitivity
PMID: 31711919
Disease Associationsⓘ20
goutOpen Targets
0.15Weak
neuroendocrine neoplasmOpen Targets
0.11Weak
Abnormal pupillary functionOpen Targets
0.07Suggestive
diabetes mellitusOpen Targets
0.03Suggestive
chronic kidney diseaseOpen Targets
0.03Suggestive
oral squamous cell carcinomaOpen Targets
0.02Suggestive
hepatocellular carcinomaOpen Targets
0.02Suggestive
ovarian dysfunctionOpen Targets
0.02Suggestive
Increased total eosinophil countOpen Targets
0.02Suggestive
neoplasmOpen Targets
0.02Suggestive
spondylolisthesisOpen Targets
0.02Suggestive
cancerOpen Targets
0.01Suggestive
lung cancerOpen Targets
0.01Suggestive
non-small cell lung carcinomaOpen Targets
0.01Suggestive
acute myeloid leukemiaOpen Targets
0.01Suggestive
T-cell acute lymphoblastic leukemiaOpen Targets
0.01Suggestive
arrhythmogenic right ventricular cardiomyopathyOpen Targets
0.00Suggestive
cardiomyopathyOpen Targets
0.00Suggestive
hypertrophic cardiomyopathyOpen Targets
0.00Suggestive
osteosarcomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PABIR3Shared pathway33%PABIR2Shared pathway33%PSMD10Shared pathway25%DET1Shared pathway25%DDX49Shared pathway25%SUMO2Shared pathway25%
Tissue Expression6 tissues
Brain
100%
Heart
77%
Liver
71%
Ovary
68%
Lung
62%
Bone Marrow
54%
Gene Interaction Network
Click a node to explore
PABIR1PABIR3PABIR2PSMD10DET1DDX49SUMO2
PROTEIN STRUCTURE
Preparing viewer…
PDB8TWE · 2.55 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.41Moderately Constrained
pLIⓘ
0.99Intolerant
Observed/Expected LoF0.13 [0.05–0.41]
RankingsWhere PABIR1 stands among ~20K protein-coding genes
  • #11,204of 20,598
    Most Researched34
  • #2,073of 17,882
    Most Constrained (LOEUF)0.41 · top quartile
Genes detectedPABIR1
Sources retrieved9 papers
Response time—
📄 Sources
9▼
1
CHK1 Inhibitor Blocks Phosphorylation of FAM122A and Promotes Replication Stress.
PMID: 33108758
Mol Cell · 2020
1.00
2
Cryo-EM structures of PP2A:B55-FAM122A and PP2A:B55-ARPP19.
PMID: 38123684
Nature · 2024
0.89
3
FAM122A ensures cell cycle interphase progression and checkpoint control by inhibiting B55α/PP2A through helical motifs.
PMID: 38982062
Nat Commun · 2024
0.78
4
FAM122A Inhibits Erythroid Differentiation through GATA1.
PMID: 32763160
Stem Cell Reports · 2020
0.67
5
FAM122A maintains DNA stability possibly through the regulation of topoisomerase IIα expression.
PMID: 32866497
Exp Cell Res · 2020
0.56